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Behçet’s disease, also known as Behçet’s syndrome, is a long-term auto-inflammatory disorder that affects the body’s blood vessels.

This can lead to many different symptoms, including vascular problems, oral sores, genital and skin sores, eye inflammation, arthritis, and inflammation of the gut, brain, and spinal cord.

It is a relapsing and remitting condition, which means that sometimes there may be no symptoms, but, during a flare, symptoms worsen for a while.

epidemiology-

Behçet’s disease is seen all over the world. But it’s most common in Northern Turkey (up to 420 cases per 100,000 people), the Mediterranean basin and Middle East (up to 300 cases per 100,000 people), and the Far East (about 15 cases per 100,000 people).

It appears that Behçet’s disease in India is predominantly ‘mucocutaneous’ and ‘arthritic’; ‘ocular’ and ‘neuro’ Behçet’s being uncommon. In comparison to published literature, the onset of disease in this part of the world is significantly delayed. The pathergy test is rarely positive.

Pathophysiology

Theories behind the pathogenesis of Behçet disease currently suggest an autoimmune etiology.It is thought that in genetically predisposed individuals, exposure to an infectious agent or an environmental antigen triggers the autoimmune response.

Infectious triggers

Exposure to an infectious agent may trigger a cross-reactive immune response. Proposed infectious agents have included the following:

• Herpes simplex virus (HSV)
• Streptococcus species
• Staphylococcus species
• Escherichia coli

The International Study Group for Behçet’s Disease has emphasized the presence of recurrent oral ulcers as a primary consideration in the diagnosis of Behçet disease.  In response, the pathogens above have been targeted for study, with the hope of establishing a direct link between their presence and disease activity. Unfortunately so far, researchers have been unable to generalize results across geographic populations.

The study of heat shock proteins (HSPs) has provided some insight into possible mechanisms that contribute to the development of Behçet disease. Through discovery that human HSP-60 and HSP-65 share greater than 50% homology with mycobacterial HSP, enhanced T-cell response has been elicited with exposure to both bacterial and human homogenates in Behçet disease patients compared with controls in United Kingdom, Japanese, and Turkish populations.

HSP-65, found in high concentrations in oral ulcers and active skin lesions in patients with Behçet disease, has also been demonstrated to stimulate production of antibodies that exhibit cross-reactivity with streptococcal species present in the mouth.  Feng et al suggested that HSP-A6 levels may be useful in differentiating intestinal Behçet disease from Crohn disease: in their study, serum HSP-A6 expression was significantly elevated in intestinal Behçet disease (0.72 ± 0.39 ng/mL) compared with Crohn disease (0.50 ± 0.24 ng/mL, P = 0.000) and healthy controls (0.38 ± 0.37 ng/mL). The clinical relevance of enzyme-linked immunosorbent assay (ELISA) testing to measure HSPA6 is currently unknown.

Attempts at determining whether tissue antigens have a role in channeling the immune response have been unsuccessful. Elevated peripheral levels of gamma-delta T cells (γδ⁺ T cells) in patients with Behçet disease in response to exposure to mycobacterial HSPs compared with those in healthy subjects imply a role for their production.  Antigen-driven expansion of oligoclonal Vβ⁺ T-cell receptor (TCR)–specific cell lines in Behçet disease patients has been demonstrated.  However, generalization of these results is not applicable because of the high degree of interindividual variability in TCR expression.

T cells and neutrophils

Systemic involvement of multiple organs is observed in Behçet disease, rooted primarily in the development of vasculitic or vasculopathic lesions in the affected areas. These areas may demonstrate microscopic evidence of inflammatory tissue infiltration with both T cells and neutrophils. 

Studies of T lymphocytes have suggested a T-helper type 1 (TH1)–predominant response. Both CD4⁺ and CD8⁺ lymphocytes demonstrate higher concentrations in peripheral blood, with characteristic and corresponding elevations of cytokines (interleukin [IL]–2] and interferon-γ [IFN-γ]). Serum levels of IL-12 have also been shown to be elevated in patients with Behçet disease, possibly helping drive the response. Decreased levels and impaired activity of natural killer cells were demonstrated in bronchoalveolar lavage specimens of Behçet disease patients with pulmonary manifestations.

Because of the degree of neutrophilic infiltration demonstrated in characteristic Behçet disease lesions (eg, hypopyon, pustular lesions, and pathergy reactions), the activity and function of these cells has been explored extensively. Unfortunately, existing studies offer inconsistent results regarding cell adhesion and chemotactic behavior, superoxide production, and phagocytic properties.

Thus, the specific role of neutrophils in Behçet disease has been difficult to characterize. Some studies have found that cytokine release in Behçet disease may, by an unknown mechanism, place neutrophils in a static pre-excitatory “primed” state, eventually triggered into hyperactivity by environmental stimuli at a lower threshold than in individuals who do not have Behçet disease. 

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Genetics

Behçet disease is a sporadic disease, but a familial aggregation is well known.  Carriers of HLA-B51/HLA-B5 have an increased risk of developing Behçet disease compared with noncarriers.  HLA-B51 is the the strongest associated genetic factor and it has been shown to be more prevalent in Turkish, Middle Eastern, and Japanese populations, corresponding with a higher prevalence of Behçet disease in these populations. However, HLA-B51 has not been shown to affect the severity of symptoms and is less prevalant in patients not from endemic areas. 

In addition, genome-wide association (GWA) studies have linked increased susceptibility to Behçet disease with polymorphisms in genes encoding for cytokines, activator factors, and chemokines.   Specific single-nucleotide polymorphisms involving the following genes have been identified  :

• Familial Mediterranean fever gene ( MEFV) mutation Met694Val
• TLR4 (involved in pathogen recognition and activation of innate immunity)
• ERAP1 (codes for a molecule that processes microbial proteins in white blood cells)
• CCR1-CCR3 (involved in recruitment of effector immune cells to sites of infection)
• STAT4 (involved in increased risk for autoimmune disease)
• KLRK1-KLRC4

Symptoms

Behcet’s disease symptoms vary from person to person, can come and go or become less severe over time. Signs and symptoms depend on which parts of your body are affected.

Areas commonly affected by Behcet’s disease include:

• Mouth. Painful mouth sores that look similar to canker sores are the most common sign of Behcet’s disease. They begin as raised, round lesions in the mouth that quickly turn into painful ulcers. The sores usually heal in one to three weeks, though they do recur.
• Skin. Some people develop acnelike sores on their bodies. Others develop red, raised and tender nodules on their skin, especially on the lower legs.
• Genitals. Red, open sores can occur on the scrotum or the vulva. The sores are usually painful and can leave scars.
• Eyes. Inflammation in the eye (uveitis) causes redness, pain and blurred vision, typically in both eyes. In people with Behcet’s disease, the condition can come and go.
• Joints. Joint swelling and pain often affect the knees in people with Behcet’s disease. The ankles, elbows or wrists also might be involved. Signs and symptoms can last one to three weeks and go away on their own.
• Blood vessels. Inflammation in veins and arteries can cause redness, pain, and swelling in the arms or legs when a blood clot results. Inflammation in the large arteries can lead to complications, such as aneurysms and narrowing or blockage of the vessel.
• Digestive system. A variety of signs and symptoms can affect the digestive system, including abdominal pain, diarrhea and bleeding.
• Brain. Inflammation in the brain and nervous system can cause headache, fever, disorientation, poor balance or stroke.

When to see a doctor

Make an appointment with your doctor if you notice unusual signs and symptoms that might indicate Behcet’s disease. If you’ve been diagnosed with the condition, see your doctor if you notice new signs and symptoms.

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Causes

The exact cause of Behçet’s disease is unknown, but it is thought to be an autoimmune disease.

In this type of condition, the immune system mistakenly reacts to a normal substance or process in the body, leading to symptoms of inflammation.

Certain groups of people have a higher risk of developing the disease than others.

Age: All ages and sexes are at risk of developing Behçet’s disease, but it most commonly affects people in their 20s and 30s. Men typically experience more severe symptoms than women.

Ethnicity and geographic location: These may play a role in how likely a person is to develop Behçet’s disease. The condition is most common in men from the Middle East and Asia, and women from the United States, other Western Countries, Japan, and Korea.

Genetic and hereditary factors: There may be a genetic or inherited component to the disease. Behçet’s disease could also be linked to bacteria, viruses, or environmental factors. More research is needed, however, before these suggestions can be confirmed.

Risk factors

Factors that might increase your risk of Behcet’s include:

• Age. Behcet’s disease commonly affects men and women in their 20s and 30s, though children and older adults also can develop the condition.
• Where you live. People from countries in the Middle East and East Asia, including Turkey, Iran, Japan and China, are more likely to develop Behcet’s.
• Sex. While Behcet’s disease occurs in both men and women, the disease is usually more severe in men.
• Genes. Having certain genes is associated with a higher risk of developing Behcet’s.

Complications

Complications of Behcet’s disease depend on your signs and symptoms. For instance, untreated uveitis can lead to decreased vision or blindness. People with eye signs and symptoms of Behcet’s disease need to visit an eye specialist (ophthalmologist) regularly because treatment can help prevent this complication.

Diagnosis

Because there is not a single test to diagnose Behçet’s disease, doctors need to rule out any conditions that mimic the disease.

The International Clinical Criteria for Behçet’s disease diagnosis require that certain symptoms must be present for a diagnosis to be made.

A diagnosis requires:

• The presence of recurring mouth ulcers at least three times in one single year

In addition to the above, at least two of the criteria below must also be met:

• recurring genital ulcers
• eye inflammation (uveitis) confirmed by an eye exam
• skin sores in adults who are not taking corticosteroids
• a positive pathergy test reading within 24-48 hours of the test

In a pathergy test, a doctor inserts a small, clean needle into the skin of the forearm. A positive result is given if a small, red bump forms 1 to 2 days after the needle has been inserted.

History

In 1990, the International Study Group (ISG) for Behçet’s Disease clarified criteria for the diagnosis of Behçet disease.  The ISG group compared the clinical findings of 914 patients with a history of aphthous ulcers with those of controls. Initial criteria for diagnosis require the occurrence of at least three episodes of oral herpetiform or aphthous ulcerations within a 12-month period observed directly by a physician or reported by the patient. To confirm the diagnosis, at least two of the following must also be demonstrated:

• Recurrent painful genital ulcers that heal with scarring
• Ophthalmic lesions, including anterior or posterior uveitis, hypopyon, or retinal vasculitis
• Skin lesions, including erythema nodosum–like lesions, pseudofolliculitis, or papulopustular or acneiform lesions
• Positive results from pathergy skin testing, defined as the formation of a sterile erythematous papule 2 mm in diameter or larger that appears 48 hours following a skin prick with a sharp sterile needle (22-24 gauge [a dull needle may be used as a control])

See the Behcet’s Syndrome International Study Group Criteria calculator.

Considering the above diagnostic criteria, case presentation often includes the following characteristics:

• Multiorgan system involvement, often beginning with mucocutaneous involvement and usually sparing the liver, kidneys, and heart
• Age of 25-35 years at onset
• Organ-specific manifestations characterized by exacerbations and a relapsing/remitting course

Skin and mucous membrane manifestations

Painful oral lesions (aphthous or herpetiform) are one of the criteria for diagnosis and may be the first manifestation (70% of cases). See the image below.

Oral aphthous ulcers secondary to Behçet disease.

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Oral lesions are commonly found in keratinized areas of the oropharynx, often excluding the nonkeratinized surfaces of the dorsal tongue, gums, and hard palate. The lesions are usually not distinguishable from those due to other causes but often have a high recurrence rate (often more than five times per year, despite only three times per year specified in ISG criteria) and appear as multiple lesions or crops (often more than six simultaneous lesions at a given time).

Skin lesions often occur in the genital region of both sexes. In males, scrotal involvement is most characteristic; however, lesions can also develop on the penile shaft. In females, the labial area is most commonly involved, with lesions occasionally developing in the vagina and on the perineum. Genital ulcerations typically heal with scarring and are more painful in men. Development of ulcerations in women may correlate with menstruation.

Nodules that resemble erythema nodosum are more common in the lower extremities of females. They are tender, erythematous, and nodular and usually resolve after 2-3 weeks but often recur. Erythema nodosum may be an indicator of mild Behçet disease.

Acneiform papulopustular lesions are more common in men and are usually found on the trunk and extremities, although they may develop anywhere on the body.

Extragenital ulcerations that heal with scarring are rare and affect only 3% of patients.  These are very specific for Behçet disease. They can be found in the axillae, neck, breast, interdigital skin of the feet, and groin.

Positive pathergy test is more common in Turkish and Japanese populations, as well as patients with ophthalmic and neurologic manifestations.

Ocular lesions

Ocular presentations (anterior or posterior uveitis, hypopyon, retinal vasculitis, cystoid macular degeneration) represent the first manifestation of disease in 10% of patients with Behçet disease but usually occur following oral ulceration. 

Symptoms commonly include blurred vision, periorbital pain, photophobia, scleral injection, and excessive lacrimation.

Men, particularly of Iranian and Japanese descent, tend to present with more severe eye involvement.

Highly recurrent posterior uveitis can lead to blindness.

Ocular symptoms usually present in the first years of illness. Cases that cause blindness commonly develop within the first 7 years. The prognosis is better for persons who develop symptoms later in the disease course.

Neurologic manifestations

Collectively, neurologic signs and symptoms tend to be an unusual late manifestation, 1-8 years after disease onset. They include the following:

• Memory tends to be affected in most cases, particularly affecting recall and learning. Orientation, arithmetic, and language are often unaffected.
• Symptoms are usually parenchymal in nature, predominantly with brainstem involvement. 
• Behavioral changes, primarily apathy or disinhibition, occur in 54% of patients.
• Seizures and bulbar signs with ophthalmoplegia are less common.

Other less common findings include the following:

• Infarctions due to vasculitis or thrombosis
• Meningoencephalitis
• Lymphocytic meningeal infiltration
• Demyelinization

Vasculopathy

Behçet disease can cause aneurysms in the pulmonary arterial tree that often prove to be fatal. Pulmonary artery aneurysmal involvement is associated with right-sided cardiac thromboses and can manifest as hemoptysis, cough, chest pain, or dyspnea. 

Vasculitis of the small and large vessels can cause a panoply of symptoms depending on location of the lesions.

Arterial disease predominantly affects males and only rarely occurs in women. 

Venous involvement (usually in the form of superficial thrombophlebitis) is more common than arterial involvement.  Superficial thrombophlebitis presents in a linear fashion with overlying erythema and is often confused with erythema nodosum. In males, formation of these linear areas of vasculopathy leads to sclerosis and stringlike thickening in the affected areas.

Symptoms correlate with the vessel involved and may be devastating. For example, extension of an inferior vena caval clot to the hepatic vein may be the mechanism of Budd-Chiari syndrome in Behçet disease.

Arthritis

Arthritis and arthralgias occur in as many as 60% of patients and primarily affect the lower extremities, especially the knee. Ankles, wrists, and elbows can also be primarily involved.

The arthritis is nondeforming and asymmetric in nature and can assume a monoarticular, oligoarticular, or polyarticular pattern of involvement.

Symptoms relapse and remit and rarely become chronic.

Diffuse arthralgias are also common.

Gastrointestinal (GI)/genitourinary manifestations (GU)

GI involvement affects 3-16% of patients with Behçet disease. Areas affected often include the esophagus and ileocecal area. Symptoms include abdominal pain, bloating, and GI bleeding. Complications often result from deep ulceration of intestinal sections.

GU involvement can include epididymitis, neurogenic bladder, and sterile urethritis. Neurogenic bladder can present with typical symptoms of urinary retention.

Renal manifestations

Renal manifestations may be underreported. One study found that 1-29% of patients with Behçet disease developed such manifestations. 

Associated amyloidosis may develop.

The first presentation is often nephritic-range proteinuria found incidentally. Crescenteric and proliferative glomerulonephritis, as well as IgA nephritis, have also been reported in some cases. 

Other manifestations

Cardiac manifestations (5-17% of cases) include the following::

• Coronary vasculitis and thrombosis
• Pericarditis
• Myocarditis
• Endocarditis with granulomatous changes or fibrosis
• Regurgitation
• Diastolic dysfunction

Lung involvement occurs in up to 18% of patients with Behçet disease. Pulmonary vasculitis, hypertension, and pleural effusions have been reported. Aneurysms represent a dreaded complication of Behçet disease and may result in massive hemoptysis. The detection of a pulmonary aneurysm in the setting of a vasculitic illness is highly suggestive of Behçet disease. 

Drug therapies

According to the United Kingdom’s National Health Service (NHS), medications include:

Corticosteroids: These reduce inflammation and may be used as a systemic treatment, affecting the whole body, or in topical applications, for example, to treat mouth sores.

Immunosuppressants: These reduce are systemic medications the excessive activity of the immune system, which underlies most of the symptoms of Behçet’s.

Biological therapies: This is a newer, systemic therapy. It targets some of the specific biological processes that are involved in causing symptoms. For example, tumour necrosis factor alpha inhibitors (TNFa-inhibitors) affect the antibodies that lead to inflammation.

Topical therapy

This is applied to the surfaces of the body. It may include the use of pain-relieving therapy, including corticosteroid rinses, gels, eye drops, and ointments. Examples include triamcinolone acetonide, betamethasone, and dexamethasone.

Oral therapy

At times, it may be necessary to undergo treatment with drugs that work throughout the body. These drugs include:

• colchicine, a medication used to prevent gout
• corticosteroids
• medications to suppress the immune system such as azathioprine, cyclosporine, and cyclophosphamide
• medications that change how the body’s immune cells work

Additional medications may be recommended based on the symptoms that develop. Patients should discuss treatment options with their healthcare provider.

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During pregnancy

Behçet’s does not appear to be linked to pregnancy complications, but the medications used can be harmful to the unborn baby.

For this reason, it is best for any pregnancy to be planned and discussed first with a health provider.

Sometimes a baby is born with neonatal Behçet’s disease. This is very rare and usually resolves itself within 6 to 8 weeks.

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Reactive arthritis is joint pain and swelling triggered by an infection in another part of the body — most often the intestines, genitals or urinary tract.

This condition usually targets the knees, ankles and feet. Inflammation also can affect the eyes, skin and the tube that carries urine out of the body (urethra). Previously, reactive arthritis was sometimes called Reiter’s syndrome.

Reactive arthritis isn’t common. For most people, signs and symptoms come and go, eventually disappearing within 12 months.

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Causes

Reactive arthritis develops in reaction to an infection in your body, often in your intestines, genitals or urinary tract. You might not be aware of the triggering infection if it causes mild symptoms or none at all.

Numerous bacteria can cause reactive arthritis. Some are transmitted sexually, and others are foodborne. The most common ones include:

• Campylobacter
• Chlamydia
• Clostridioides difficile
• Escherichia coli
• Salmonella
• Shigella
• Yersinia

Reactive arthritis isn’t contagious. However, the bacteria that cause it can be transmitted sexually or in contaminated food. Only a few of the people who are exposed to these bacteria develop reactive arthritis.

Pathophysiology

ReA is usually triggered by a GU or GI infection (see Etiology). Evidence indicates that a preceding Chlamydia respiratory infection may also trigger ReA.  The frequency of ReA after enteric infection averages 1-4% but varies greatly, even among outbreaks of the same organism. Although severely symptomatic GI infections are associated with an increased risk of ReA, asymptomatic venereal infections more frequently cause this disease.  About 10% of patients have no preceding symptomatic infection.

ReA is associated with histocompatibility leukocyte antigen B-27 (HLA-B27), a major histocompatibility complex (MHC) class I molecule involved in T-cell antigen presentation. Results for HLA-B27 are positive in 65-96% of patients (average, 75%) with ReA. Patients with HLA-B27, as well as those with a strong family clustering of the disease, tend to develop more severe and long-term disease.

Sun et al reported that susceptibility to ReA arthritis is affected by the levels of certain killer cell immunoglobulin-like receptors (KIRs), which correspond with specific HLA-C ligand genotypes. In individuals with high levels of activating and low levels of inhibitory KIR signals, pathogens can more easily trigger natural killer cell and T cell innate and adaptive immune responses, resulting in the overproduction of cytokines that contribute to the pathogenesis of ReA.

Their study of 138 patients with ReA found that KIR2DS1, which is activating, is associated with susceptibility to ReA, when present alone or in combination with the HLA-C1C1 genotype. KIR2DL2, which is inhibitory, in combination with the HLA-C1 ligand is associated with protection against ReA. Patients with ReA had significantly lower frequencies of KIR2DL2 and KIR2DL5 than did controls. The presence of more than seven inhibitory KIR genes was protective. 

The mechanism by which the interaction of the inciting organism with the host leads to the development of ReA is not known. It is possible that microbial antigens cross-react with self-proteins, stimulating and perpetuating an autoimmune response mediated by type 2 T helper (Th2) cells. Chronicity and joint damage have been associated with a Th2 cytokine profile that leads to decreased bacterial clearance.

Synovial fluid cultures are negative for enteric organisms or Chlamydia species. However, a systemic and intrasynovial immune response to the organisms has been found with intra-articular antibody and bacterial reactive T cells. Furthermore, bacterial antigen has been found in the joints. Thus, the elements for an immune-mediated synovitis are present.

Synovitis in ReA is mediated by proinflammatory cytokines. Native T cells under the influence of transforming growth factor (TGF)-β and other cytokines, such as interleukin (IL)-6, differentiate into Th17 effector cells, which then produce IL-17. IL-17 is one of the major cytokines elevated in the synovial fluid of these patients.  Deficiencies in regulatory mechanisms can result in increased proinflammatory cytokine production and worse outcome. 

The Toll-like receptors (TLRs) recognize different extracellular antigens as part of the innate immune system.  TLR-4 recognizes gram-negative lipopolysaccharide (LPS). Studies in mice and humans showed abnormalities in antigen presentation due to downregulation of TLR-4 costimulatory receptors in patients with ReA. Subsequent studies implicated TLR-2 polymorphism associated with acute ReA; however, its role is still disputed. 

Molecular evidence of bacterial DNA (obtained via polymerase chain reaction [PCR] assay) in synovial fluids has been found only in Chlamydia -related ReA, and a single placebo-controlled trial of a tetracycline derivative (ie, lymecycline) has shown a reduction in the duration of acute Chlamydia -related, but not enteric-related, ReA.  This suggests that persistent infection may play a role, at least in some cases of chlamydial-associated ReA.

In a subsequent trial, the combination of doxycycline and rifampin was superior to doxycycline alone in reducing morning stiffness and swollen and tender joints in patients with undifferentiated spondyloarthropathy. 

The role of HLA-B27 in this scenario remains to be fully defined. The following theories have been proposed:

• Molecular mimicry – This hypothesis suggests that a similarity exists at the molecular level between the HLA-B27 molecule and the inciting organisms, allowing the triggering of an immune response and the subsequent development of clinical disease 
• HLA-B27 as a receptor for certain bacteria – At present, there is little evidence either to confirm or to refute this hypothesis
• Defective class I antigen-mediated cellular response – This hypothesis suggests that the HLA-B27 molecule may be a defective molecule associated with an aberrant cytotoxic T-cell response

ReA can occur in patients with HIV infection or AIDS—most likely because both conditions can be sexually acquired, rather than because ReA is triggered by HIV. The course of ReA in these patients tends to be severe, with a generalized rash resembling psoriasis, profound arthritis, and frank AIDS. HLA-B27 frequency is the same as that associated with non–AIDS-related ReA in a similar demographic group. This association points out the likely importance of CD8⁺ cytotoxic T cells as compared with CD4⁺ Th cells in the pathogenesis of ReA.

ReA is sometimes divided into epidemic and endemic forms. Whereas a triggering agent can be identified for epidemic ReA, none has been identified for endemic ReA. Differentiation between the 2 types of ReA may be difficult in some cases; however, it is not essential to either diagnosis or treatment.

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epidemiology

International statistics

The infections that incite ReA may vary with geographic location. For example, Y enterocolitica is more commonly identified in Europe than in North America and thus is responsible for more cases of ReA in countries such as Finland and Norway. The occurrence of ReA appears to be related to the prevalence of HLA-B27 in a population and to the rate of urethritis/cervicitis and infectious diarrhea.

More than 40 subtypes of HLA-B27 are known; those associated with the spondyloarthropathies are HLA-B2702, B2704, and B2705.  These subtypes may be somewhat geographically segregated. For example, the subtype B2705 is found predominantly in Latin America, Brazil, Taiwan, and parts of India. It is noteworthy that subtypes HLA-B2706 and B2709—found in native Indonesia and Sardinia, respectively—may be partially protective against ReA. 

In Norway, an annual incidence of 4.6 cases per 100,000 population for chlamydial ReA and an incidence of 5 cases per 100,000 population for enteric bacteria–induced ReA were reported in 1988-1990. In Finland, nearly 2% of males were found to have ReA after nongonococcal urethritis; the incidence of HLA-B27 is higher in the Finnish population. In the United Kingdom, the incidence of ReA after urethritis is about 0.8%. In the Czech Republic, the annual incidence of ReA in adults during 2002-2003 was reported at 9.3 cases per 100,000 population. 

clinical presentation

History

Reactive arthritis (ReA) usually develops 2-4 weeks after a genitourinary (GU) or gastrointestinal (GI) infection (or, possibly, a chlamydial respiratory infection . About 10% of patients do not have a preceding symptomatic infection. The classic triad of symptoms—noninfectious urethritis, arthritis, and conjunctivitis—is found in only one third of patients with ReA and has a sensitivity of 50.6% and a specificity of 98.9%.  In postenteric ReA, diarrhea and dysenteric syndrome (usually mild) is commonly followed by the clinical triad in 1-4 weeks.

In a large percentage of ReA cases, conjunctivitis or urethritis occurred weeks before the patient seeks medical attention. Patients may fail to mention this unless specifically asked. Musculoskeletal disease is evident in many of these patients. Vague, seemingly unrelated complaints may obscure this diagnosis at times. 

The onset of ReA is usually acute and characterized by malaise, fatigue, and fever. An asymmetrical, predominantly lower-extremity, oligoarthritis is the major presenting symptom. Myalgias may be noted early on. Asymmetric arthralgia and joint stiffness, primarily involving the knees, ankles, and feet (the wrists may be an early target), may be noted. Low-back pain occurs in 50% of patients.  Heel pain associated with Achilles enthesopathies and plantar fasciitis is also common.

Both postvenereal and postenteric forms of ReA may manifest initially as nongonococcal urethritis, with frequency, dysuria, urgency, and urethral discharge; this urethritis may be mild or inapparent. Urogenital symptoms, whether resulting from GU infection or from GI infection, are found in 90% of patients with ReA. 

An estimated 0.5-1% of cases of urethritis evolve into ReA. Urethritis develops acutely 1-2 weeks after infection through sexual contact and is similar to gonococcal urethritis. A purulent or hemopurulent exudate appears, and the patient complains of dysuria.

In men, chlamydial urethritis is less painful and produces less purulent discharge than acute gonorrhea does, making it difficult to notice. Findings from microscopic examination and cultures can be used to rule out Neisseria gonorrhoeae infection. Coinfection with Chlamydia and Neisseria organisms is common in some areas. In women, urethritis and cervicitis may be mild, with dysuria or slight vaginal discharge, or asymptomatic, which makes diagnosis difficult.

Often, the initial urethritis is treated with antibiotics (especially wide-spectrum tetracyclines or macrolides) when findings suggest gonorrhea. Despite an apparent early cure, the manifestations of the disease appear several weeks later, and the patient may not relate them to a previous episode of urethritis.

Lymphogranuloma venereum infection may be asymptomatic; screening should be considered in all men who have sex with men (MSM) presenting with acute arthritis, particularly if they are infected with HIV. 

In addition to conjunctivitis, ophthalmologic symptoms of ReA include erythema, burning, tearing, photophobia, pain, and decreased vision (rare).

Patients may have mild recurrent abdominal complaints after a precipitating episode of diarrhea.

Association with HIV infection

ReA is particularly common in the context of HIV infection. Accordingly, patients with new-onset ReA must be evaluated for HIV. The existing immunodepression in patients with AIDS poses special management problems.

HIV-positive ReA patients are at risk for severe psoriasiform dermatitis, which commonly involves the flexures, scalp, palms, and soles. Frequently, psoriasiform dermatitis is associated with arthritis that involves the distal joints in a destructive pattern. The disturbances of immune homeostasis in AIDS could account for this peculiar expression of psoriasis in these patients.

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Risk factors

Certain factors increase your risk of reactive arthritis:

• Age. Reactive arthritis occurs most frequently in adults between the ages of 20 and 40.
• Sex. Women and men are equally likely to develop reactive arthritis in response to foodborne infections. However, men are more likely than are women to develop reactive arthritis in response to sexually transmitted bacteria.
• Hereditary factors. A specific genetic marker has been linked to reactive arthritis. But most people who have this marker never develop the condition.

Prevention

Genetic factors appear to play a role in whether you’re likely to develop reactive arthritis. Though you can’t change your genetic makeup, you can reduce your exposure to the bacteria that may lead to reactive arthritis.

Store your food at proper temperatures and cook it properly. Doing these things help you avoid the many foodborne bacteria that can cause reactive arthritis, including salmonella, shigella, yersinia and campylobacter. Some sexually transmitted infections can trigger reactive arthritis. Use condoms to help lower your risk.

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treatment

Approach Considerations

No curative treatment for reactive arthritis (ReA) exists. Instead, treatment aims at relieving symptoms and is based on symptom severity. Almost two thirds of patients have a self-limited course and need no treatment other than symptomatic and supportive care. As many as 30% of patients develop chronic symptoms, posing a therapeutic challenge.

Physical therapy, nonsteroidal anti-inflammatory drugs (NSAIDs), and intralesional corticosteroids may be helpful for joint, tendon, and fascial inflammation. Low-dose prednisone may be prescribed, but prolonged treatment is not advisable. Antibiotics may be given to treat underlying infection. Disease-modifying antirheumatic drugs (DMARDs) such as sulfasalazine and methotrexate may be used safely and are often beneficial. No specific surgical treatment is indicated.

Hospitalization of a patient with uncomplicated ReA is not usually indicated. Inpatient care may be considered for patients who are unable to tolerate oral administration of medications, who are unable to ambulate because of significant joint involvement, who have intractable pain, or who have concomitant disease necessitating admission.

Few treatment options exist for HIV-infected patients with severe ReA. Treatment of ReA in the setting of HIV infection poses special problems. However, potentially immunosuppressive therapies (eg, cyclosporine, methotrexate, and psoralen plus ultraviolet A [PUVA]) have been used in some cases, with variable success and relatively few severe complications. A case report from the United Kingdom suggests that antiretroviral therapy may be considered in HIV-infected ReA patients who are unresponsive to standard therapy. 

No dietary limitations are necessary unless the patient is receiving steroid therapy. Efforts should be made to maintain joint function with physical activity, joint protection, and suppression of inflammation. Physical therapy may be instituted to avoid muscle wasting and to reduce pain in severe cases. Although no limitations on physical activity need be imposed, symptoms of arthritis will usually limit patients’ activity to some extent.

Pharmacologic Therapy

NSAIDs (eg, indomethacin and naproxen) are the foundation of therapy for ReA.  Etretinate/acitretin has been shown to decrease the required dosage of NSAIDs.  Sulfasalazine or methotrexate may be used for patients who do not experience relief with NSAIDs after 1 month or who have contraindications to NSAIDs. In addition, sulfasalazine-resistant ReA may be successfully treated with methotrexate.

In a series of 22 pediatric ReA patients from the Republic of China, NSAIDs and sulfasalazine were the mainstays of treatment, with cyclophosphamide used in 14 patients and methotrexate and corticosteroids added in a few. Most achieved full remission within 6 months.

Antibiotic treatment is indicated for cervicitis or urethritis but generally not for postdysenteric ReA. In Chlamydia-induced ReA, some data suggest that prolonged combination antibiotic therapy could be an effective treatment strategy. 

Case reports exist that demonstrate the effectiveness of anti−tumor necrosis factor (TNF) medications,  such as etanercept and infliximab. No published data are available on the effectiveness of selective cyclooxygenase (COX)–2 inhibitors; however, a COX-2 inhibitor may be tried in patients who do not tolerate NSAIDs and in whom no preexisting contraindication to COX-2 use exists.

Symptom-specific approaches

Arthritis and enthesitis

Joint symptoms are best treated with aspirin or other short-acting and long-acting anti-inflammatory drugs (eg, indomethacin, naproxen). In one report, a patient became asymptomatic after 3 months of aspirin at a dosage of 80 mg/kg/day; the dosage was gradually reduced and eventually discontinued. A combination of NSAIDs is reportedly effective in severe cases. No published data suggest that any NSAID is more effective or less toxic than another (controlled treatment trials are difficult to conduct with an uncommon disease).

Varying success in treating severe cases of ReA with other medications (eg, sulfasalazine, methotrexate, etretinate, ketoconazole, azathioprine, or intra-articular steroid injections) has been reported. In a refractory case or a patient with HIV-associated ReA, the anti−TNF-α agent infliximab may be successful.  Depending on the culture results, a short course of antibiotics may be needed; however, treatment may not affect the disease course. Longer-term administration of antibiotics to treat joint symptoms provides no established benefits.

Conjunctivitis and uveitis

Transient and mild conjunctivitis is usually not treated. Mydriatics and cycloplegics (eg, atropine) with topical corticosteroids may be administered in patients with acute anterior uveitis. Patients with recurrent ocular involvement may require systemic corticosteroid therapy and immunomodulators to preserve vision and prevent ocular morbidity. 

Urethritis and gastroenteritis

Antibiotics may be considered for urethritis and gastroenteritis, depending on the cultures used and their sensitivity. In general, urethritis may be treated with a 7- to 10-day course of erythromycin or tetracycline. Antibiotic treatment of enteritis is controversial.

Mucocutaneous lesions

Only local care is necessary for mucosal lesions. Topical steroids may be needed for psoriasiform lesions; the use of hydrocortisone or triamcinolone may be beneficial. A topical keratolytic, such as 10% salicylic acid ointment, can be added if needed. Topical salicylic acid and hydrocortisone with oral aspirin has also been suggested. 

Hydrocortisone 2.5% cream and salicylic acid 10% ointment are effective in treating chronic keratoderma blennorrhagicum and circinate balanitis, though either condition may heal without medical treatment. Circinate balanitis usually responds to topical steroids; however, it can be recurrent and create a therapeutic challenge. Balanitis refractory to conventional therapy can be successfully treated with the complementary use of topical 0.1% tacrolimus. 

Systemic therapy, if required, consists of the administration of oral acitretin, PUVA, methotrexate, cyclosporine, or some combination thereof.

Nonsteroidal anti-inflammatory drugs

The choice of a specific NSAID depends on the individual response to treatment. Phenylbutazone may work in patients refractive to other NSAIDs. These agents should be used regularly to achieve a good anti-inflammatory effect. Patients must be instructed on compliance and the possible need to adjust the dosage or switch to another agent. Treatment must be continued for 1 month at maximum dosage before effectiveness can be fully evaluated.

NSAIDs may reduce the intensity and the frequency of recurrences of ocular inflammation and allow a decrease in the corticosteroid dosage, which helps decrease the chances of cataract formation and other associated corticosteroid effects.

The decreased awareness of pain sometimes seen with the use of NSAIDs may alter the patient’s recognition of recurrences. Patients should be examined whenever any change in symptoms occurs to evaluate for recurrence of an acute episode of inflammation. Ocular involvement may parallel systemic and joint disease relapses.

Corticosteroids

Corticosteroids may be given either via intra-articular injection or as systemic therapy. For ocular manifestations of ReA, they may also be given topically.

Joint injections can produce long-lasting symptomatic improvement and help avoid the use of systemic therapy. Sacroiliac joints can be injected, usually under fluoroscopic guidance. 

Systemic corticosteroids may be particularly useful in patients who do not respond well to NSAIDs or who experience adverse effects related to the use of NSAIDs. The starting dose is guided by a patient’s symptoms and objective evidence of inflammation. Prednisone can be used initially at a dosage of 0.5-1 mg/kg/day, tapered according to response.

Topical corticosteroids and mydriatics should be used early and aggressively to reduce tissue damage. Prolonged topical treatment is necessary for several weeks after the inflammation has cleared; early withdrawal of topical corticosteroids frequently results in the return of inflammatory changes. Keratolytics or topical corticosteroids may improve cutaneous lesions. Topical corticosteroids may be useful for iridocyclitis.

Antibiotics

The current view of the pathogenesis of ReA indicates that an infectious agent is the trigger of the disease, but antibiotic treatment does not change the course of the disease, even when a microorganism is isolated. In these cases, antibiotics are used to treat the underlying infection, but specific treatment guidelines for ReA are lacking.

However, in Chlamydia -induced ReA, studies have suggested that appropriate treatment of the acute genitourinary (GU) infection can prevent ReA and that treatment of acute ReA with a 3-month course of tetracycline reduces the duration of illness. Empiric antibiotics may be considered after appropriate cultures have been taken. Nongonococcal urethritis and other infections can be treated specifically with systemic antibiotics. In the absence of contraindications, treatment of urethritis is recommended, even if improvement is not certain.

Although urethritis and cervicitis are commonly treated with antibiotics, diarrhea generally is not. No evidence indicates that antibiotic therapy benefits enteric-related ReA or chronic ReA of any cause.

Long-term antibiotic therapy may be warranted in cases of poststreptococcal ReA; however, this is currently a controversial topic.

Lymecycline (a tetracycline available outside the United States) was studied in a double-blind placebo-controlled study of patients with chronic ReA for a treatment period of 3 months.  The duration of illness was significantly shorter in patients with Chlamydia -induced disease than in those with disease triggered by enteric infections.

Azithromycin was shown to be ineffective in a placebo-controlled trial. Nevertheless, in another study, azithromycin or doxycycline in combination with rifampin for 6 months was reported to be significantly superior to placebo and significantly improved symptoms associated with Chlamydia-induced ReA.

Quinolones have been studied because of their broad coverage, but no clear benefit has been reported. In a randomized, double-blind, placebo-controlled study of 56 patients with recent-onset ReA, 3 months of treatment with a combination of ofloxacin and roxithromycin was not better than placebo in improving outcomes.

More studies are needed before definite recommendations can be made for the role of antibiotics in the management of ReA. 

Disease-modifying antirheumatic drugs

In patients who have chronic symptoms or have persistent inflammation despite the use of the agents mentioned above, other second-line drugs may be used. Clinical experience with these DMARDs has been mostly in rheumatoid arthritis and in psoriatic arthritis. However, DMARDs have also been used in ReA, though their disease-modifying effects in this setting are uncertain.

Sulfasalazine has been shown to be beneficial in some patients. The use of this drug in ReA is of interest because of the finding of clinical or subclinical inflammation of the bowel in many patients. Sulfasalazine is more widely used in ankylosing spondylitis. In a 36-week trial of sulfasalazine versus placebo to treat spondyloarthropathies, patients with ReA who were taking sulfasalazine had a 62.3% response rate, compared with a 47.7% rate for the placebo group in peripheral arthritis. 

Methotrexate may be used in patients who present with rheumatoidlike disease. Several reports have shown good response, but controlled studies are lacking. Reports also describe the use of azathioprine and bromocriptine in ReA, but again, large studies have not been published. Patients with ReA who have HIV infection or AIDS should not receive methotrexate or other immunosuppressive agents.

Case reports have demonstrated the effectiveness of anti-TNF medications, such as etanercept and infliximab, though there remains a need for randomized, double-blind trials. The high concentrations of TNF-α in the serum and joints of patients with persistent ReA suggest that this cytokine could be targeted in patients who do not respond to NSAIDS and DMARDs. Anti−TNF-α therapy has been demonstrated to be effective treatment for ReA, with a corticosteroid-sparing effect. 

However, TNF-α antagonists can increase the risk of serious infection, and it is important to conduct infectious screening and monitoring with a high index of suspicion, as well as preemptive treatment, when such medications are used.  Anti-TNF medications can also be associated with severe glomerulonephritis, and it is recommended that renal function be closely monitored in patients treated with these agents. 

Interleukin (IL)-6 plays an important role in regulating immune response. Unregulated overproduction of IL-6, however, is pathologically involved in various immune-mediated inflammatory diseases, including ReA. Tocilizumab, a humanized anti–IL-6 receptor antibody, may provide clinical benefit in patients who are refractory to conventional therapy or anti-TNF therapy.  However, further clinical studies are required.

Surgical Intervention

No surgical therapy for ReA is recommended. However, surgical intervention may be warranted for certain ocular manifestations of the disease.

The posterior spillover of inflammatory material in the chronic iridocyclitis associated with ReA may result in persistent vitreous opacification. The cumulative effects of secondary involvement of the vitreous may result in visually disabling vitreous debris and opacification, making these eyes good candidates for vitrectomy. Although vitrectomy should be considered only after prolonged follow-up care and thorough planning, it appears to offer a definitive improvement in vision in certain cases.

Because of the intense episodes of recurrent inflammation, it is essential to render the eyes as quiet as possible before surgery by using topical, periocular, or systemic corticosteroids. At least 3 months of cell-free slit lamp examinations—6 months for younger patients and severe cases—should be documented before elective surgical intervention.

Preoperative ultrasonography is helpful in determining the degree of vitreous opacification, the thickening of the choroid, and the presence of a cyclitic membrane, which can create significant problems at surgery.

The major objective of surgery in patients with complicated uveitic cataract and vitreous opacification is to improve vision. Vitrectomy may favorably modify the dynamics of the uveitic process, though lensectomy-vitrectomy does not reduce the inflammatory reaction in all cases.

Cystoid macular edema is the major cause of decreased visual acuity after surgery; however, this is a common and serious complication of chronic uveitis even without surgery. Vitrectomy may actually reduce cystoid macular edema with gradual resolution over 1 year and an improvement in vision in some patients.

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Sjogren’s (SHOW-grins) syndrome is a disorder of your immune system identified by its two most common symptoms — dry eyes and a dry mouth.

The condition often accompanies other immune system disorders, such as rheumatoid arthritis and lupus. In Sjogren’s syndrome, the mucous membranes and moisture-secreting glands of your eyes and mouth are usually affected first — resulting in decreased tears and saliva.

Although you can develop Sjogren’s syndrome at any age, most people are older than 40 at the time of diagnosis. The condition is much more common in women. Treatment focuses on relieving symptoms.

epidemiology

Internationally, comparative studies between different ethnic groups have suggested that Sjögren syndrome is a homogeneous disease that occurs worldwide with similar prevalence and affects 1-2 million people.

The female-to-male ratio of Sjögren syndrome is 9:1. Sjögren syndrome can affect individuals of any age but is most common in elderly people. Onset typically occurs in the fourth to fifth decade of life.

Symptoms

The two main symptoms of Sjogren’s syndrome are:

• Dry eyes. Your eyes might burn, itch or feel gritty — as if there’s sand in them.
• Dry mouth. Your mouth might feel like it’s full of cotton, making it difficult to swallow or speak.

Some people with Sjogren’s syndrome also have one or more of the following:

• Joint pain, swelling and stiffness
• Swollen salivary glands — particularly the set located behind your jaw and in front of your ears
• Skin rashes or dry skin
• Vaginal dryness
• Persistent dry cough
• Prolonged fatigue

Causes

Sjogren’s syndrome is an autoimmune disorder. Your immune system mistakenly attacks your body’s own cells and tissues.

Scientists aren’t certain why some people develop Sjogren’s syndrome. Certain genes put people at higher risk of the disorder, but it appears that a triggering mechanism — such as infection with a particular virus or strain of bacteria — is also necessary.

In Sjogren’s syndrome, your immune system first targets the glands that make tears and saliva. But it can also damage other parts of your body, such as:

• Joints
• Thyroid
• Kidneys
• Liver
• Lungs
• Skin
• Nerves

Prognosis

Sjögren syndrome carries a generally good prognosis. In patients who develop a disorder associated with Sjögren syndrome, the prognosis is more closely related to the associated disorder (eg, SLE, lymphoma). Interestingly, primary Sjögren syndrome is associated with lower cardiovascular risk factors and lower risk of serious cardiovascular events such as myocardial infarction and stroke, in comparison with SLE.​ 

Although salivary and lacrimal function generally stabilize, the presence of SSA and/or hypocomplementemia may predict a decline in function.

Morbidity and mortality

Morbidity associated with Sjögren syndrome is mainly associated with the gradually decreased function of exocrine organs, which become infiltrated with lymphocytes. The increased mortality rate associated with the condition is primarily related to disorders commonly associated with Sjögren syndrome, such as SLE, RA, and primary biliary cirrhosis. Patients with primary Sjögren syndrome who do not develop a lymphoproliferative disorder have a normal life expectancy. 

Risk factors

Sjogren’s syndrome typically occurs in people with one or more known risk factors, including:

• Age. Sjogren’s syndrome is usually diagnosed in people older than 40.
• Sex. Women are much more likely to have Sjogren’s syndrome.
• Rheumatic disease. It’s common for people who have Sjogren’s syndrome to also have a rheumatic disease — such as rheumatoid arthritis or lupus.

Complications

The most common complications of Sjogren’s syndrome involve your eyes and mouth.

• Dental cavities. Because saliva helps protect the teeth from the bacteria that cause cavities, you’re more prone to developing cavities if your mouth is dry.
• Yeast infections. People with Sjogren’s syndrome are much more likely to develop oral thrush, a yeast infection in the mouth.
• Vision problems. Dry eyes can lead to light sensitivity, blurred vision and corneal damage.

Less common complications might affect:

• Lungs, kidneys or liver. Inflammation can cause pneumonia, bronchitis or other problems in your lungs; lead to problems with kidney function; and cause hepatitis or cirrhosis in your liver.
• Lymph nodes. A small percentage of people with Sjogren’s syndrome develop cancer of the lymph nodes (lymphoma).
• Nerves. You might develop numbness, tingling and burning in your hands and feet (peripheral neuropathy).

History

The clinical presentation of Sjögren syndrome may vary. Most patients are women, and onset is usually at age 40-60 years, but the syndrome also can affect men and children. The onset is insidious. The first symptoms in primary Sjögren syndrome can be easily overlooked or misinterpreted, and diagnosis can be delayed for as long as several years.

Xerophthalmia (dry eyes) and xerostomia (dry mouth) are the main clinical presentations in adults. Bilateral parotid swelling is the most common sign of onset in children.

Extraglandular involvement in Sjögren syndrome falls into two general categories: periepithelial infiltrative processes and extraepithelial extraglandular involvement. Periepithelial infiltrative processes include interstitial nephritis, liver involvement, and bronchiolitis and generally follow a benign course.

Extraepithelial extraglandular involvement in Sjögren syndrome is related to B-cell hyperreactivity, hypergammaglobulinemia, and immune complex formation and includes palpable purpura, glomerulonephritis, and peripheral neuropathy. These latter manifestations occur later in the course of Sjögren syndrome and are associated with a higher risk of transformation to lymphoma.

Symptoms of Sjögren syndrome can decrease the patient’s quality of life in terms of its physical, psychological, and social aspects.

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Sicca symptoms (dry eyes and dry mouth)

Although dry eyes and dry mouth are the most common symptoms in patients with Sjögren syndrome, most patients who report these symptoms have other underlying causes. The incidence of sicca symptoms increases with age. Indeed, more than one third of elderly persons have sicca symptoms. Whether this is part of the normal aging process (associated with fibrosis and atrophy observed on some lip biopsy studies) or is due to the accumulation of associated illnesses and medications is unclear. 

Common medications that can cause sicca symptoms in any age group include antidepressants, anticholinergics, beta blockers, diuretics, and antihistamines. Anxiety can also lead to sicca symptoms. Women who use hormone replacement therapy may be at increased risk of dry eye syndrome. 

Patients may describe the effects dry mouth in the following ways:

• Inability to eat dry food (eg, crackers) because it sticks to the roof the mouth
• Tongue sticking to the roof of the mouth
• Putting a glass of water on the bed stand to drink at night (and resulting nocturia)
• Difficulty speaking for long periods of time or the development of hoarseness
• Higher incidence of dental caries and periodontal disease
• Altered sense of taste
• Difficulty wearing dentures
• Development of oral candidiasis with angular cheilitis, which can cause mouth pain

Dry eyes may be described as red, itchy, and painful. However, the most common complaint is that of a gritty or sandy sensation in the eyes. Symptoms typically worsen throughout the day, probably due to evaporation of the already scanty aqueous layer. Some patients awaken with matting in their eyes and, when severe, have difficulty opening their eyes in the morning. Blepharitis can also cause similar morning symptoms.

Parotitis

Patients with Sjögren syndrome may have a history of recurrent parotitis, often bilateral. Although in some patients the parotid glands become so large that the patients report this as a problem, more often the examining physician discovers them.

Cutaneous symptoms

Nonvasculitic cutaneous manifestations in Sjögren syndrome include the following  :

• Dryness
• Eyelid dermatitis
• Pruritus
• Erythema annulare

Cutaneous vasculitis, such as palpable purpura, develops in some patients with Sjögren syndrome, especially those with hypergammaglobulinemia or cryoglobulinemia.  Raynaud phenomenon is observed in approximately 20% of patients.

Pulmonary symptoms

Patients with Sjögren syndrome can develop dryness of the tracheobronchial mucosa (xerotrachea), which can manifest as a dry cough.  Less often, patients develop dyspnea from an interstitial lung disease that is typically mild. Patients may develop recurrent bronchitis or even pneumonitis (infectious or noninfectious).

Gastrointestinal symptoms

Dryness of the pharynx and esophagus frequently leads to difficulty with swallowing (deglutition), in which case patients usually describe food becoming stuck in the upper throat.  Lack of saliva may lead to impaired clearance of acid and may result in gastroesophageal reflux and esophagitis.

Abdominal pain and diarrhea can occur. Rarely, patients develop acute or chronic pancreatitis, as well as malabsorption due to pancreatic insufficiency. However, caution is advised when interpreting laboratory results because an elevated amylase level may arise from the parotid gland.

Patients with gastritis should be tested for Helicobacter pylori infection, because of its association with gastric mucosa–associated lymphoid tissue lymphomas.

Patients with Sjögren syndrome are at increased risk for delayed gastric emptying, which can cause early satiety, upper abdominal discomfort, nausea, and vomiting. 

Cardiac symptoms

Pericarditis and pulmonary hypertension, with their attendant symptomatology, can occur in Sjögren syndrome.  Orthostatic symptoms related to dysfunction of autonomic control of blood pressure and heart rate is associated with increased severity of Sjögren syndrome. 

Neurologic symptoms

The occurrence of central nervous system (CNS) and spinal cord involvement in Sjögren syndrome is estimated by various studies to be 8-40%, with manifestations including myelopathy, optic neuropathy, seizures, cognitive dysfunction, and encephalopathy.  Attempts must be made to distinguish other causes of these symptoms, including concomitant SLE, multiple sclerosis, cerebrovascular disease, and Alzheimer disease.

Sensory, motor, or sensorimotor peripheral neuropathy, often subclinical, can be detected in up to 55% of unselected patients with Sjögren syndrome.  Symptoms of distal paresthesias may be present. Cranial neuropathies can develop, particularly trigeminal neuropathy or facial nerve palsy. Mononeuritis multiplex should prompt a search for a vasculitis.

Progressive weakness and paralysis secondary to hypokalemia due to underlying renal tubular acidosis can occur and is potentially treatable. 

Renal symptoms

Renal calculi, renal tubular acidosis, and osteomalacia, nephrogenic diabetes insipidus, and hypokalemia can occur secondary to tubular damage caused by interstitial nephritis, the most common form of renal involvement in Sjögren syndrome.

Interstitial cystitis, with symptoms of dysuria, frequency, urgency, and nocturia, is strongly associated with Sjögren syndrome. 

Glomerulonephritis can be caused by Sjögren syndrome but is uncommon and is usually attributable to another disorder, such as SLE or mixed cryoglobulinemia.

Additional symptoms

Nasal dryness can result in discomfort and bleeding. Women may also have a dry vagina, which can lead to dyspareunia, vaginitis, and pruritus.

Patients with Sjögren syndrome may report fatigue, joint pain, and, sometimes, joint swelling. A careful review of systems must be performed to differentiate these from the manifestations of other disorders (see DDx). Fibromyalgia is common in patients with Sjögren syndrome, with a prevalence of about 31%. 

Women with Sjögren syndrome may have a history of recurrent miscarriages or stillbirths, and women and men may have a history of venous or arterial thrombosis. These are related to the presence of antiphospholipid antibodies (eg, lupus anticoagulant or anticardiolipin antibodies).

Secondary Sjogren syndrome

Secondary Sjögren syndrome appears late in the course of the primary disease. However, in some patients, primary Sjögren syndrome may precede SLE by many years. Secondary Sjögren syndrome is usually mild, and sicca symptoms are the main feature. Unlike patients with primary Sjögren syndrome, persons with the secondary type have significantly fewer systemic manifestations. These manifestations include the following:

• Salivary gland swelling
• Lung involvement
• Nervous system involvement
• Renal involvement
• Raynaud phenomenon
• Lymphoproliferative disorders

In secondary Sjögren syndrome, symptoms of the primary disease predominate. Secondary Sjögren syndrome does not modify the prognosis or outcome of the basic disease. Polyarteritis nodosa and Sjögren syndrome may also coexist, perhaps best viewed as an overlap syndrome. 

Approach Considerations

No curative agents for Sjögren syndrome exist. The treatment of the disorder is essentially symptomatic.

Skin and vaginal dryness

Patients should use skin creams, such as Eucerin, or skin lotions, such as Lubriderm, to help with dry skin. Vaginal lubricants, such as Replens, can be used for vaginal dryness. Vaginal estrogen creams can be considered in postmenopausal women. Watch for and treat vaginal yeast infections.

Arthralgias and arthritis

Acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs) can be taken for arthralgias. Consider hydroxychloroquine if NSAIDs are not sufficient for the synovitis occasionally associated with primary Sjögren syndrome. However, hydroxychloroquine does not relieve sicca symptoms. Patients with RA associated with Sjögren syndrome likely require other disease-modifying agents.

Additional treatment considerations

In patients with major organ involvement, such as lymphocytic interstitial lung disease, consider therapy with steroids and immunosuppressive agents, such as cyclophosphamide.

While cyclophosphamide and similar agents may be helpful for treating serious manifestations of Sjögren syndrome or disorders associated with Sjögren syndrome, clinicians should understand that these agents are also associated with the development of lymphomas.

Long-term anticoagulation may be needed in patients with vascular thrombosis related to antiphospholipid antibody syndrome.

In a small group of patients with primary Sjögren syndrome, mycophenolate sodium reduced subjective, but not objective, ocular dryness and significantly reduced hypergammaglobulinemia and RF.

Among the biologic therapies, the greatest experience in primary Sjögren syndrome is with rituximab, an anti-CD20 (which is expressed on B-cell precursors) monoclonal antibody. Anti-B–cell strategies, particularly rituximab, have a promising effect in the treatment of patients with severe extraglandular manifestations of Sjögren syndrome.

Reports on the use of rituximab in patients with primary Sjögren syndrome have emerged in the literature. In a double-blind, randomized, placebo-controlled trial, Meijer et al found that rituximab significantly improved saliva flow rate, lacrimal gland function, and other variables in patients with primary Sjögren syndrome.

In an open-label clinical trial, modest improvements were noted in patient-reported symptoms of fatigue and oral dryness. However, no significant improvement in the objective measures of lacrimal and salivary gland function was noted, despite effective depletion of blood B cells. In a randomized, placebo-controlled, parallel-group study of 120 patients with primary Sjögren syndrome, treatment with rituximab did not alleviate disease activity or symptoms at week 24, although it did alleviate some symptoms at weeks 6 and 16. 

Rituximab appears promising in the treatment of vasculitis and intravenous immunoglobulin (IVIG)–dependent ataxic neuropathy. Results from the AIR registry (French) indicated that rituximab appears to be effective in cryoglobulinemia or vasculitis-related peripheral nervous system involvement in primary Sjögren syndrome.

In a prospective study of 78 patients with primary Sjögren syndrome treated with rituximab, significant improvement in extraglandular manifestations was reported, as measured by EULAR [European League Against Rheumatism] Sjögren Syndrome Disease Activity Index (ESSDAI) (disease activity score) and overall good tolerance reported.  Several smaller studies of rituximab revealed improvement of arthralgias, regression of parotid gland swelling, and improvement of immune-related thrombocytopenia.

Of the TNF inhibitors, both etanercept and infliximab have failed to demonstrate significant benefit in Sjögren syndrome.

Combination therapy with leflunomide and hydroxychloroquine resulted in a significant decrease in ESSDAI scores and caused no serious adverse events, in a small phase 2a randomized clinical trial from the Netherlands. At 24 weeks, the mean difference in ESSDAI score in the leflunomide-hydroxychloroquine group (n=21), compared with the placebo group (n=7), was –4.35 points after adjustment for baseline values.

Fewer data are available with regard to the role of anti-CD22, anti-BAFF, anti-IL-1, type 1 interferon, and anti-T–cell agents in treatment of primary Sjögren syndrome, with further investigations ongoing. The overall paucity of evidence in therapeutic studies in primary Sjögren syndrome suggests that much larger trials of the most promising therapies are necessary. The investigators concluded that further evaluation of leflunomide–hydroxychloroquine combination therapy in larger clinical trials is warranted.

Emergency department care

The diagnosis of Sjögren syndrome can be made from the ED if the index of suspicion is high. Patients may present with mild symptoms (eg, eye grittiness, eye dryness or discomfort, dry mouth, recurrent caries). Bilateral parotid gland swelling is also a common presentation.

Patients with known Sjögren syndrome should not be taken lightly for their complaint of dry eyes or dry mouth, as these chronic problems can be very distressing and obtrusive.

Inpatient care

Give attention to artificial lubricants and humidified oxygen for intubated and/or sedated patients with Sjögren syndrome.

Outpatient care

Encourage patients with Sjögren syndrome to be active. In addition, patients should be encouraged to avoid exacerbation of dryness symptoms (eg, through smoking or exposure to low-humidity environments). All patients with Sjögren syndrome should be monitored by an ophthalmologist and dentist, in addition to their rheumatologist. Certain patients may be candidates for punctal occlusion, which is usually performed by an ophthalmologist.

Monitoring

Most patients with Sjögren syndrome can be monitored at follow-up visits every 3 months and, if the patient is stable, up to every 6 months. Patients with active problems or in whom an emerging associated illness is a concern can be seen as often as monthly.

Surgical Therapy

Occlusion of the lacrimal puncta can be corrected surgically. Electrocautery and other techniques can be used for permanent punctal occlusion.

During surgery, the anesthesiologist should administer as little anticholinergic medication as possible and use humidified oxygen to help avoid inspissation of pulmonary secretions. Good postoperative respiratory therapy should also be provided. Patients are at higher risk for corneal abrasions, so ocular lubricants should be considered.

Biopsies that may be performed in association with Sjögren syndrome include the following:

• Minor salivary gland biopsy – For diagnostic purposes
• Parotid gland biopsy – If malignancy is suggested
• Biopsy of an enlarged lymph node – To help rule out pseudolymphoma or lymphoma

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Acute disseminated encephalomyelitis (ADEM) is a rare kind of inflammation that affects the brain and spinal cord, usually in children. It damages the coating that protects nerve fibers, called myelin.

Symptoms may be severe, but they can be treated. Most people make a full recovery and don’t have another attack.

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated inflammatory demyelinating condition that predominately affects the white matter of the brain and spinal cord. The disorder manifests as an acute-onset encephalopathy associated with polyfocal neurologic deficits and is typically self-limiting.  ADEM bears a striking clinical and pathological resemblance to other acute demyelinating syndromes (ADS) of childhood, including multiple sclerosis (MS). ADEM in children is readily distinguishable from alternative diagnoses on the basis of clinical features and findings on neuroimaging and laboratory investigations. However, given that ADEM lacks a specific identified biological marker rendering a reliable laboratory diagnosis, long-term follow-up is important as there are instances where an illness initially diagnosed as ADEM is ultimately replaced with a diagnosis of MS. 

The onset of ADEM usually occurs in the wake of a clearly identifiable febrile prodromal illness or immunization and in association with prominent constitutional signs and encephalopathy of varied degrees. ADEM is typically a monophasic disease of pre-pubertal children; whereas, MS is typically a chronic relapsing and remitting disease of young adults. Abnormalities of findings on cerebrospinal fluid (CSF) immunoglobulin studies are less common in ADEM. However, the division between these processes is indistinct, suggesting a clinical continuum. Moreover, other conditions along the suggested continuum include optic neuritis, transverse myelitis, and neuromyelitis optica – clinical entities that may occur as manifestations of either MS or ADEM. ^[2] Other boundaries of ADEM merge indistinctly with a wide variety of inflammatory encephalitic and vasculitic illnesses as well as monosymptomatic, postinfectious illnesses that should remain distinctfromADEM, such as acute cerebellar ataxia (ACA). A furtherindistinct boundary is shared by ADEM and Guillain-Barré syndrome as manifested in cases of Miller-Fisher syndrome and encephalomyeloradiculoneuropathy (EMRN).

Susceptibility to either ADEM or MS is likely the product of multiple factors, including a complex interrelationship of genetics and exposure to infectious agents and other environmental factors. Of particular interest are the indications that susceptibility to either condition is in part age-related. Most cases of ADEM possibly occur as the result of an inflammatory response provoked by pre-pubertal infection with a virus, vaccine, or other infectious agent. Typically, the manifestations of ADEM occur quickly after this pre-pubertal febrile systemic illness and are monophasic. In a minority of cases, patients with ADEM experience one or two pre-pubertal recurrences followed by remission. MS, on the other hand, typically manifests as a relapsing-remitting illness in ensuing adolescence or young adulthood, a significant and unexplained latency of effect with apparent permanency of immune dysregulation. Bouts of MS occur without a febrile prodrome. Uncommonly, MS develops in pre-pubertal individuals andADEMdevelops in post-pubertal individuals. In very rare instances, individuals manifest pre-pubertal ADEM and, after long latency, MS in adolescence.

epidemiology

Few studies have provided incidence data from other countries, thus little is known about occurrence throughout the world. Genetic factors, prevalence of infectious pathogens, immunization status, degree of skin pigmentation, diet, and other factors may influence risk.

History

Clinically, acute disseminated encephalomyelitis (ADEM) is usually readily distinguishable from multiple sclerosis (MS) by the presence of certain clinical features, including the following:

• History of preceding infectious illness or immunization, although a clear preceding event may be absent in up to a quarter of patients. 
• Association with constitutional symptoms and signs, such as fever
• Prominence of cortical signs such as mental status changes and seizures
• Comparative rarity of posterior column abnormalities, which are common in MS
• Age younger than 11-12 years in ADEM and age older than 11-12 years in MS

ADEM is more common in the winter months, with most cases occurring between October and March. Typical cases of ADEM arise 1-2 days to several weeks after a childhood infectious illness.

• There is usually a clearly defined phase of afebrile improvement lasting 2-21 days or more before onset of neurologic findings.
• Generally, patients have shown partial or complete recovery from the prodromal illness at the time of onset of ADEM.
• Whether latencies of longer than 21 days implicate a particular febrile illness as the prodrome of ADEM is unclear. Clinical experience suggests that this is possible.
• Most of the large envelope-bearing viruses that figured prominently in older series of ADEM, of which measles was a particularly virulent example, no longer figure importantly in the etiology of ADEM because these diseases are prevented by vaccination.
• Most cases encountered now occur in the wake of respiratory or gastrointestinal illness presumed to be of viral etiology, although a specific virus is seldom identified.
• Documentation of at least 1 fever-free day is especially suggestive of ADEM, although such a hiatus is also found in post-infectious vasculitides.
• Occasionally, ADEM may occur in the wake of several weeks of fever of unknown origin.
• Some patients have premonitory pain in the back prior to the development of ADEM-related inflammatory myelitis.
• Various vaccines have been suggested as the exogenous provocation of cases of ADEM.
  • This remains a controversial subject, although clear evidence exists for the role of the Pasteur rabies vaccine and compelling, although somewhat less conclusive evidence exists for the role of other vaccines.
  • The overall effect of the introduction of vaccinations for measles and other encephalomyelitogenic viruses has been a marked reduction in the number of severe or fatal cases of ADEM.
  • Measles was associated with ADEM in about 1 out of 800 cases, and in many of these cases, ADEM that was often particularly severe. Measles-associated ADEM had a high rate of both morbidity and mortality.
• A cause-and-effect relationship between a possible prodrome and ADEM is more difficult to establish in cases where longer or very short intervals exist between a possible exogenous stimulus and inflammatory result.
  • Latencies longer than 50 days have been suggested for infections or vaccines but are difficult to prove. 
  • Relationships are also difficult to determine when a febrile systemic process is rapidly followed by neurologic deterioration because such cases may represent meningoencephalitis.
    • Approximately 25% of cases lack a clearly-defined prodrome.
    • Some of these cases are possible examples of longer than 20 days of latency from prodrome to ADEM, especially in prepubertal children, with imaging changes suggesting ADEM, with negative CSF immune profile, and with rapid and complete recovery.
    • Another subgroup with poorly-defined prodrome but low risk for recurrence are children or adolescents manifesting subacute-onset syndromes that combine neuropsychiatric abnormalities and movement disorders and imaging changes suggestive of ADEM. The course in these cases, which could be termed Johnson syndrome, is often prolonged or even progressive, improving with high-dose intravenous corticosteroids.

The first signs of ADEM usually include abrupt onset encephalopathy (alteration in consciousness or behavioral change unexplained by fever, systemic illness or postictal symptoms. Rapid-onset encephalopathy is typically associated with multifocal neurologic symptoms.

• In most cases, the clinical course is rapidly progressive and typically develops over hours to maximum deficits within days (mean of 4.5 days). A minority of cases show continued deterioration of function for periods as long as 4 weeks.
• Strictly speaking, encephalopathy, unexplained by fever, should be present for a diagnosis of ADEM, though it may not be the presenting sign. A single institution follow-up study (at least 5.5 y for each individual) of 52 young individuals (age range 10 mo to 19 y) who presented with their first bout of an acute central nervous system demyelinating disease included 26 children ultimately diagnosed with MS and 24 diagnosed with ADEM. Encephalopathy was the presenting sign in 42% of those with a follow-up diagnosis of ADEM but none of the individuals with a follow-up diagnosis of MS. 
• Convulsive seizures occur around the onset of ADEM in as many as 35% of cases.
• Meningismus may be present and has been reported in up to 30% of cases.
• Although almost any portion of the CNS may be clinically involved, certain systems appear to be particularly prone to dysfunction; thus, the descending white matter motor tracts, optic nerves, and spinal cord are particularly commonly involved.
• ADEM-associated optic neuritis is typically bilateral, although the onset in a second eye may follow onset in the first by days to months. Bilaterality may provide a degree of reassurance with regard to MS risk as optic neuritis in MS is frequently unilateral. Visual evoked responses may discern abnormalities in a second eye before clinical deterioration in vision is discernible.
  • A wide variety of cranial nerve abnormalities may occur in addition to optic nerve disease.
  • Long tract signs (eg, clonus, increased muscle stretch reflexes, upgoing toes) are present early in as many as 85% of cases.
  • In some instances, reflexes may be lost at the onset. When this is caused by transverse myelitis, the evolution of disease after spinal shock replaces absent reflexes with increased muscle stretch reflexes within a few days or more. A small number of cases manifest loss of reflexes as a sign of associated peripheral nerve disease with ADEM, a condition termed EMRN. Some of these EMRN cases are associated with evidence for acute infection with Epstein-Barr virus.
    • Weakness may be hemiparetic, double hemiparetic, diparetic, or generalized and symmetric. Fairly symmetric leg weakness is seen in many cases of ADEM-related transverse myelitis with associated abnormalities of bowel and bladder function.

Some ADEM presentations are fulminant.

• Fulminant ADEM is more likely to manifest in children younger than 3 years, with rapid evolution of a low state of function and demonstration of severe edema on neuroimaging. Such cases have become uncommon with widespread vaccination against childhood illnesses.
• Transverse myelitis (TM) may begin rapidly and be associated with severe edema, usually in the cervical region. ADEM-related TM must be distinguished from TM associated with MS, vascular accidents, and directly infectious conditions, including enterovirus. It must also be distinguished from neuromyelitis optica (NMO), which may present with TM in isolation. NMO is a condition for which a biological marker (anti-AQP4 IgG in serum and/or CSF) has been identified.
  • Child/adolescent NMO represents approximately 5% of cases of NMO. Onset is a median range of 10-14 y and the vast majority of these patients are girls or young women. The median number of spinal levels involved is 10 vertebral segments.  Motor signs are usually more prominent than sensory signs. CNS lesions may be demonstrated on scans and mental status changes may be noted.
• Acute administration of very high-dose intravenous corticosteroids may possibly close the blood-brain barrier and subtend the development of edema, which may, in these fulminant cases, account for the high risk for permanent morbidity.

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There are unusual presentations for possible ADEM that have uncertain classification. More literature is supporting a continuum of acute demyelinating diseases in childhood and adulthood.

• Cases of pediatric patients diagnosed with neuromyelitis optica presenting with a clinical and radiographic evidence of ADEM have been reported. 
• Cases of patients with anti-NMDA receptor encephalitis and ADEM-like lesions on MRI have also been reported. 
• Additionally, pediatric cases of ADEM followed by recurrent or monophasic optic neuritis have been described. 
• Young children may manifest a rapidly progressive demyelinating illness that may be fatal within days to weeks and is almost universally associated with profound permanent psychomotor deficits in those who survive. Brain images differ from those typical of juvenile MS and may demonstrate confluent symmetric areas (butterfly pattern) of bright signal abnormality on T2-weighted sequences.
  • Fulminant presentation with lesions showing significant degrees of ring enhancement after contrast administration may also be found.
  • Malignant brain edema may be present, manifested by sulcal and ventricular effacement.
• Some patients with the large tumor-like lesions, acute MS, or Schilder disease presentations during childhood or adolescence do remarkably well as compared to adults with similar presentations.
• The classification of rare severe infantile cases, exhibiting features suggesting either severe acute MS or hyperacute ADEM, remains in doubt.
  • Nonetheless, pathological confirmation that some of these cases are MS has been published, and hyperacute adult cases with similar clinical and radiographic manifestations have been reported. 
  • Some of these cases display more generalized T2-weighted abnormalities on MRI and may represent cases of what has been referred to as acute toxic encephalopathy.
  • Emphasizing that scan results do not reliably distinguish every case of MS from ADEM is important, but in most cases, reliable inferences may be drawn. Extensive white matter involvement may be found in young infants that some would label as MS  while others would label it hyperacute ADEM.
• Rarely, childhood, adolescent, or adult MS manifests as large unilateral or multiple tumor-like mass lesions that may appear cystic and may impart mass effects (albeit atypically and, if present, unexpectedly mildly). The lesions are steroid responsive and may recur in other locations, such as the contralateral paraventricular white matter.
  • These lesions may represent an intermediate entity between MS and ADEM. Other differential considerations are neoplasm, systemic lupus erythematosus (SLE) and other vasculitic illnesses, progressive multifocal leukoencephalomyelitis, and Schilder myelinoclastic diffuse sclerosis.
  • Schilder disease (diffuse sclerosis) is sometimes considered an MS variant, and the uncertain diagnostic status is beyond the scope of this review. Detailed discussion of that entity is available in the Neurology section of the Medscape Reference journal.

Recurrence may occur during the taper of corticosteroid therapy initiated for ADEM. This phenomenon is not thought to represent a second or independent bout of illness; it usually responds to increasing the corticosteroid dosage and prolonging the ensuing taper.

The appearance of small new lesions on MRI within a month of presentation must also be interpreted with caution, and this may be seen in ADEM.

Although long tapers are sometimes required and more than one taper-related worsening occurs in a small number of patients, recovery is achieved within 2-12 months without further recurrence.

A rare subgroup of patients exists who cannot be weaned entirely from anti-inflammatory therapy. Most of the 8 examples one of the authors (RSR) has encountered were in boys, and the onset of illness usually occurred at age 2-6 years.

• Mental status changes, visual disturbance, and pyramidal weakness are typical findings; seizures occur in most cases.
• Imaging changes resemble those found in cases of typical ADEM (ie, multiple plaques at the grey-white junction and in deep white matter), a feature that distinguishes these cases from chronic cases considered a manifestation of Schilder disease.
• The CSF immune profile remains normal despite recurrences, although myelin basic protein may be elevated.
• The neurologic abnormalities in this group improve significantly with intravenous methylprednisolone treatment (20 mg/kg/d for 3 successive doses) followed by oral methylprednisolone (2 mg/kg/d) with slow taper to achieve alternate-day dosing.
• Trouble is encountered during the taper, each patient having a particular threshold for recurrence. In most of the authors’ cases, this threshold is encountered when the daily methylprednisolone dose is lowered to approximately 12-14 mg every other day.
• The neurologic worsening responds to higher corticosteroid doses, but this threshold effect cannot be overcome, and steroid therapy has been continued in these patients for periods as long as 8 years.
• Although prolonged daily steroid therapy is generally well tolerated, osteopenia may develop, and one of the authors’ patients developed vertebral compression fractures.

In 2007, the International Pediatric Multiple Sclerosis Study Group (IPMSSG) proposed operational definitions for the pediatric acquired demyelinating diseases (including ADEM) in attempts to improve consistency in terminology for clinical and research purposes. These guidelines were revised in 2013 and are outlined below. 

• The criteria requires that a child must meet all of the following to be accurately classified as pediatric ADEM:
  • A first, polyfocal clinical CNS event with presumed inflammatory demyelinating cause
  • Encephalopathy that cannot be explained by fever
  • No new clinical and MRI findings emerge 3 months or more after onset
  • Brain MRI is abnormal during the acute phase
  • Typical findings on brain MRI (discussed below) that include diffuse, poorly demarcated large lesions involving the cerebral white matter; T1 hypointense lesions of the white matter are rare; deep gray matter lesions may be present.

Recurrent ADEM was previously defined as a new event of ADEM with a recurrence of the initial symptoms and signs 3 or more months after the first ADEM event. Based upon the 2013 consensus criteria from IPMSSG, this entity is now included under the entity known as multiphasic ADEM.

Multiphasic ADEM

• Individuals who have experienced typical ADEM are at risk for recurrence. As many as 10% of children with an initial diagnosis of ADEM experience another ADEM attack, typically within the first 2-8 years after the initial attack. 
• Included under the entity of “multiphasic ADEM” are new events of ADEM 3 months or more after the initial attack that can be associated with new or re-emergence of prior clinical and/or MRI findings. 
  • Relapsing disease that follows a second ADEM attack is, by definition, no longer consistent with a diagnosis of multiphasic ADEM. Typically, these cases represent a chronic neuro-inflammatory disorder (such as MS or NMO).

Physical

Irritability and lethargy are common first signs of acute disseminated encephalomyelitis (ADEM). Fever returns and headache is present in up to half of cases. Meningismus is also detected in approximately one third of cases. Over the course of minutes to weeks, multifocal neurologic abnormalities develop. The interval from onset of symptoms to maximum deficit is varied but is typically seen at a mean of 4-7 days. Among the most common abnormalities are long tract signs, acute hemiparesis, cranial nerve abnormalities (including visual loss), ataxia, and mental status abnormalities. Mental status disturbances include lethargy, fatigue, confusion, irritability, obtundation, and coma. Focal or generalized seizures occur as an early sign in a minority of cases.

Weakness (roughly 75% of cases) is more commonly discerned than sensory defects. The combinations of these signs may suggest cortical, subcortical, brainstem, cranial nerve, or spinal cord localization. Long tract signs develop in more than half of all cases. Cranial nerve palsies (including vision loss) are found in a wide range of cases (23-89%) of childhood ADEM.  Mental or psychiatric disturbances, seizures, and cranial nerve palsies are significantly less common in adolescents or adults with a first or second bout of MS and in many adults with an illness labeled ADEM. Sensory changes may be underappreciated in young children; however, posterior column deficits and hemisensory changes are possibly much less common than in adult cases of ADEM or in early bouts of adolescent or adult MS. Band or girdle dysesthesia or Lhermitte’s sign are seldom if ever found in cases of childhood ADEM.

Ataxia is found in 28-65% of childhood ADEM cases,  to differ from cases of ACA because it is more commonly appendicular with nystagmus or generalized ataxia than the distinctive gait/trunk ataxia of ACA. Extrapyramidal disorders such as choreoathetosis or dystonia are sometimes observed.

Signs and symptoms found in cases of ADEM:

• Alteration in personality
• Abnormal consciousness
• Ataxia (appendicular more than axial or gait)
• Cranial nerve palsies
• Hallucinations
• Headache
• Language disturbances
• Meningeal signs
• Nystagmus
• Psychiatric abnormalities
• Optic neuritis
• Ophthalmoparesis
• Seizures, focal or generalized
• Sensory loss/dysesthesia
• Visual field deficits
• Vomiting

Causes

Acute disseminated encephalomyelitis (ADEM) may develop in the wake of a wide variety of infectious illnesses or immunizations, especially those associated with large envelope-bearing viruses. Among the agents most commonly identified by titer rise suggesting responsibility for the prodromal phase are Ebstein-Barr virus, cytomegalovirus, herpes simplex virus (HSV), and mycoplasma; however, a particular agent is identified only in a minority of ADEM cases.

ADEM is somewhat more common in the colder months of the year, during which these various viral illnesses are more prevalent. Prior to widespread immunization programs, measles was the most common associated illness. Now, most cases occur in the wake of respiratory or gastrointestinal illnesses that are presumed to be of viral etiology; specific viral agents are seldom identified.

The hiatus between onset of viral symptoms and onset of ADEM may range from 2-21 days. The two phases of illness are typically separated by a phase of recovery from fever and other constitutional manifestations of the initial infectious phase of illness. ADEM may possibly arise after intervals as long as 30 or more days after an infectious prodrome. The longer the interval between the presumed prodrome and ADEM, the less certain one can be of the etiologic association. A minority of cases lack a prodromal phase. Establishing the etiologic role of immunizations has proven controversial.

Clear links between the Pasteur rabies vaccine and ADEM have been established. Immunizations less frequently associated with ADEM include pertussis, measles,  Japanese B virus, tetanus, influenza, hepatitis B, diphtheria, rubella, pneumococcus, varicella, smallpox, poliomyelitis, and human papillomavirus. 

The provocation provided by an infectious agent likely requires participation of other genetic or immuno-experiential factors of the individual in order to give rise to ADEM. These factors likely include genetically or experientially determined aspects of immunoregulation, particularly T-helper cell function. Alves-Leon et al have found that the alleles HLA DQB1*0602, DRB1*1501, and DRB1*1503 confer genetic susceptibility to acute disseminated encephalomyelitis. 

Medical Care

Acute disseminated encephalomyelitis (ADEM) is often treated with high-dose intravenous corticosteroids, to which it appears to be responsive. One common protocol is 20-30 mg/kg/d of methylprednisolone (maximum dose of 1 g/d) for 3-5 days. Improvement may be observed within hours but usually requires several days. An oral taper for 4-6 weeks or some other interval is sometimes appended.

• Though there is conflicting data, at least two studies have presented data suggesting that steroid taper of 3 weeks or less may increase the risk of relapse in ADEM.
• Taper-related recurrence occurs in as many as 3-5% of cases and usually responds to prolongation of taper. Similar phenomena occur in other postinfectious diseases, such as Guillain-Barré syndrome or opsoclonus-myoclonus. A subset of patients manifest repeated recurrences that prevent discontinuation of corticosteroids or necessitate changing to various steroid-sparing treatments such as cyclophosphamide or beta-interferons. This rare and interesting subgroup tends to have onset of disease before 6 years of age, and despite recurrence, these children do not manifest evidence for CSF immune profile (ie, IgG index, IgG synthetic rate, oligoclonal bands) abnormality. The relationship of this group to patients with ADEM or MS or some other form of inflammatory CNS illness remains unclear.

The chief alternative therapy is intravenous immune globulin (IVIG).  It is administered as 2 g/kg intravenously as a single dose or over the course of 3-5 days. IVIG may be preferable in instances where meningo-encephalitis cannot be excluded based upon the hypothesis that corticosteroids might worsen the course of infection.

Available published information concerning efficacy is inadequate to accurately assess much concerning the impact of either form of therapy, although it appears likely that both forms of therapy increase the pace of initial recovery. Whether these forms of therapy influence times to final outcome or extent of final recovery is not known.

Theoretically, very high-dose corticosteroids (30-50 mg/kg) administered intravenously at presentation to patients with transverse myelitis may be advantageous from the vantage point of its capacity to close the blood-brain barrier and limit swelling. Marked cord swelling may account for poor outcome in some cases of transverse myelitis because of circulatory impairment and cord infarction. The same argument may hold true for severe cerebral ADEM such as tends to arise in some young children (< 3 y old) who also may have marked permanent neurologic impairments after severe ADEM.

There is as yet no convincing evidence that treatment with the combination of intravenous corticosteroids and IVIG confers any advantage in such cases, although this approach is employed by some clinicians.

Severe ADEM has also been treated, apparently successfully, with such alternative approaches as (1) combination of intravenous corticosteroids and IVIG, (2) cyclosporin, (3) cyclophosphamide, or (4) plasma exchange/plasmapheresis.  Greater understanding of trimolecular complex regulation, adhesion molecules, and inflammatory cytokines may permit development of more specific and effective ADEM therapies. The polymorphism of the human major histocompatibility complex and apparent heterogeneity of T cell response to autoantigens render this a daunting project, although anti-cytokines represent an intriguing avenue of therapeutic research.

Surgical Care

Surgical treatment for severely elevated intracranial pressure has been undertaken for cases of AHLE, hemorrhagic brain purpura, and non-Reye syndrome, examples of what have been termed obscure encephalopathies of infancy. Some of these cases were likely examples of hyperacute ADEM. Surgical interventions have ranged from placement of pressure bolts to decompression of the intracranial fossae by unroofing of the cranium. Outcome of such interventions was mixed.

Although such severe cases were regularly noted in the medical literature from the 1920s until the mid 1970s, few examples have been noted since that time. Prevalence clearly has dramatically decreased. Because these severe cases often followed measles, mumps, and other diseases for which effective vaccines have been developed and because the disappearance of such cases has followed the availability and use of such vaccines (earlier disappearance in the United States and Western Europe, subsequent disappearance in Asia and the Middle East), this change in prevalence likely reflects the removal of pathogens that are provocative of such severe forms of ADEM.

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Introduction

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Lesions to the central nervous system (brain or spinal cord) can lead to both positive and/ or negative upper motor neurone syndrome (UMNS) features:

Positive Component	Negative Component
Exaggerated Tendon Reflexes	Spastic Co-Contractions
Released Reflexes	Motor Weakness
Babinski Sign	Slowed Movements
Increased Tone	Loss of Dexterity
Clonus	Loss of Selective Motor Control
Spastic Dystonia

Spasticity is seen to be a positive feature of upper motor neuron syndrome. This is because it is due to a loss of inhibition of the lower motor neuron pathways, rather than a loss of connection to the lower motor neuron (or other pathways). This results from disordered sensorimotor control of movement due to a lesion of the upper motor neuron which regulate muscle control. Therefore, there is an imbalance of the signals between the central nervous system (CNS) and muscles, presenting as intermittent or sustained involuntary activation of muscles.

All muscles have some tone to maintain function, for example, activation of antigravity muscles to maintain sitting or standing postures. In an individual with spasticity, there is a velocity-dependent increase in muscle tone to passive movement. This creates an inability to stretch muscles or coordinate movements effectively.

This inhibition can result from central nervous system pathology, such as:

• Cerebral Palsy
• Multiple Sclerosis
• Motor neurone disease
• Stroke
• Hypoxic Brain Injury
• Traumatic Brain Injury
• Parkinson’s Disease
• Spinal cord injury
• Spinal cord compression
• Metastesis / tumour

Spasticity can have an impact on an individual’s function, affecting upper and lower limbs, as well as trunk. If this is not managed effectively, it can lead to fixed deformity contractures (changes to soft tissue), which affect skin care, comfort and hygiene, as well as complications for daily tasks.

The management of spasticity needs to be carefully considered as some patients rely on aspects of spasticity to allow them to maintain enough tone for functions such as standing or walking. Physiotherapy management may include facilitating individuals to utilise this to maintain weight bearing and ambulation to decrease their risk of developing osteoporosis, strengthen muscles, help improve circulation and promote overall mental health.

Definition

The most well-known and referenced description of spasticity is the physiological definition proposed by Lance in 1980. 

‘Spasticity is a motor disorder characterised by a velocity-dependent increase in tonic stretch reflexes (muscle tone) with exaggerated tendon jerks, resulting from hyperexcitability of the stretch reflex, as one component of the upper motor neurone syndrome’

More recently, a definition from Pandyan et al (2005)  states that spasticity is:

‘Disordered sensorimotor control, resulting, resulting from an upper motor neuron lesion (UMN), presenting as an intermittent or sustained involuntary activations of muscles .

Classification of Spasticity into various components of spasticity into sub-definitions:

1. Intrinsic Tonic Spasticity: Exaggeration of the tonic component of the stretch reflex (manifesting as increased tone),
2. Intrinsic Phasic Spasticity: Exaggeration of the phasic component of the stretch reflex (manifesting as tendon hyper-reflexia and clonus), and
3. Extrinsic Spasticity: Exaggeration of extrinsic flexion or extension spinal reflexes.

Epidemiology

Spasticity affects approximately:

• 35% of those with stroke,
• more than 90% with CP
• about 50% of TBI patients
• 40% of SCI patients
• between 37% and 78% of MS patients.

Anatomy and Etiology

A UMN injury leads to loss of inhibition downstream and hypersensitivity of the reflex arc within the spinal cord. Primary impairments from an upper motor neuron lesion (UMNL) are usually due to the disruption of supraspinal control of descending pathways that control excitatory and inhibitory influences on proprioceptive, cutaneous and nociceptive spinal reflexes.

The Inhibitory System

Corticoreticular Spinal Tract: These tracts travel with, but are separate from, the Corticospinal Tract, and are responsible for the facilitation of the inhibitory area within the medulla called the ventromedial reticular formation. Here, the Dorsal Reticulospinal Tract originates, which is responsible for an inhibitory action on both the stretch and flexor reflexes.

The Excitatory System

The bulbopontine tegmentum gives rise to the Medial Reticulospinal Tract and, acting weakly with the Vestibulospinal Tract, is excitatory to both stretch and extensor reflexes and like the Dorsal Reticulospinal Tract, is also inhibitory to the flexor reflexes.

Different Lesions and Their Presentations

The signs and symptoms between Cortical UMN Lesions and Spinal Cord UMN Lesions vary due to the location of where the disruption has taken place. 

Normal

Both the inhibitory system (Corticospinal Tract and Dorsoreticulospinal Tract) and excitatory systems (Medial Reticulospinal and Vestibulospinal Tract) are in dynamic balance and therefore the inhibition to the spinal cord is easily adjusted according to demand.

Corticospinal Tract Lesion

Although the Corticospinal Tract has an inhibitory influence on stretch and flexor reflex, the main inhibitory system produced by the Dorsal Reticulospinal Tract remains intact and therefore the balance of excitatory and inhibitory influences are maintained.

Internal Capsule Lesion

Leads to interruption of both the Corticospinal Tract and Corticoreticular Tract pathways that are responsible for the inhibitory response and some loss of inhibition to stretch and flexor stretches. The excitatory systems from both the Medial Reticulospinal and Vestibulospinal Tract are more dominant which leads to the facilitation of extensor and stretch reflexes but inhibition of flexors.

Incomplete Spinal Cord Lesion

Signs and symptoms will vary dependant on site and extent. If the inhibitory system is affected then there will be an unopposed excitatory drive to stretch and extensor reflexes with partial inhibition of flexor reflexes.

Complete Spinal Cord Lesion

Spinal reflexes are unopposed due to the complete loss of supraspinal control. Both flexor and extensor reflexes are disinhibited and therefore people may experience both flexor and extensor spasms.

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Clinical Presentation

On physical exam, hallmark findings include

• high muscle tone in muscle groups such as the shoulder adductors; elbow, wrist, and finger flexors; and forearm pronators. In the lower extremities, the increased tone is especially prominent in the hip adductors, knee flexors and extensors, and plantar flexors and invertors of the ankle. Antigravity muscles are predominantly affected.
• Patients may report difficulty with footwear if their spasticity involves constant, high tone of the extensor hallucis longus or long toe flexors.
• Spasticity varies with speed of movement (velocity dependent); meaning the faster the muscle is moved or stretched, the greater the resistance to stretch or passive elongation is felt.
• Clonus, spastic co-contractions, and spastic dystonia may be evident. i.e., Clonus is defined as an alternating muscle contraction and relaxation of the agonist and antagonist muscles. Spastic co-contractions are abnormal antagonist contractions that present during voluntary agonist effort. Spastic dystonia is a muscle contraction that is present at rest, leading to a constant clinical posture that is highly sensitive to stretch. 
• Clasp knife phenomenon: Limb initially resists movement and then suddenly gives way 
• Stroking Effect: Stroking the surface of the antagonist muscle may reduce tone in spasticity. 

Permanent loss of joint range has been known to occur 3-6 weeks after both stroke and brain injury and therefore it is important that spasticity is identified early on in the assessment in order for it to be monitored and managed as required. In a person with hemiplegia the lower limb pattern is plantar flexion and inversion of the ankle with hamstring tightness limiting knee range of motion as well as adductor spasticity. Upper limb presentation is usually shoulder adduction, internal rotation, elbow flexion, forearm pronation with wrist and elbow flexion. 

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Outcome measures

Useful objective measures to monitor changes in spasticity can include:

Subjective:

• Pain:
  • Numeric Pain Scale (e.g. for pain, comfort, stiffness)
  • Observing grimacing / pulling away
• Arm A and Arm B
• LegA

Objective:

• Resting Position: photographs, description, measuring bony landmarks
• Passive range of movement
  • Adductor Tone Rating
• Active range of movement
• Muscle strength: Oxford Scale
• Spasms:
  • Modified Penn Spasm Frequency Scale
  • Clonus and Spasm Score
• Function
  • Walking: 10m times walk
  • Transfers: Times up and go
  • Upper Limb Function: 9 hole peg test
  • Speech: Speech comprehension score 
  • Walking and Falls Score
• Muscle tone: Ashworth Scale / Modified Ashworth Scale, Tardieu (outlined below)

Ashworth Scale

The Ashworth scale is the most widely used assessment tool to measure resistance to limb movement in a clinic setting, although it is unable to distinguish between the neural and non-neural components of increased tone.

The scale is as follows:

Ashworth Scale
Grade	Description
0	No increase in muscle tone
1	Slight increase in tone giving a catch when the limb is moved
2	More marked increase in tone but limb easily moved
3	Considerable increase in tone – passive movement difficult
4	Limb is rigid in flexion or extension

Modified Ashworth Scale

Modified Ashworth Scale’ scores exhibited better reliability when measuring upper extremities than lower. The scale is as below:

Modified Ashworth Scale
Grade	Description
0	No increase in muscle tone
1	Slight increase in muscle tone, manifested by a catch and release or by minimal resistance at the end of the range of motion when the affected part(s) is moved in flexion or extension
1+	Slight increase in muscle tone, manifested by a catch, followed by minimal resistance throughout the remainder (less than half) of the ROM
2	More marked increase in muscle tone through most of the ROM, but affected part(s) easily moved
3	Considerable increase in muscle tone, passive movement difficult
4	Affected part(s) rigid in flexion or extension

Tardieu Scale

This scale quantifies muscle spasticity by assessing the response of the muscle to stretch applied at specified velocities. Grading is always performed at the same time of day, in a constant position of the body for a given limb. For each muscle group, reaction to stretch is rated at a specified stretch velocity. 

Tardieu
Velocity to Stretch
V1	As slow as possible
V2	Speed of the limb segment falling (with gravitational pull)
V3	At a fast rate (>gravitational pull)
Quality of Muscle Reaction
0	No resistance throughout passive movement
1	Slight resistance throughout,with no clear catch at a precise angle.
2	Clear catch at a precise angle followed by release
3	Fatiguable Clonus (< 10 secs) occurring at a precise angleFatiguable Clonus (< 10 secs) occurring at a precise angle
4	Unfatiguable Clonus (> 10 secs) occurring at a precise angle
5	Joint immobile
Spasticity Angle
R1	Angle of catch seen at Velocity V2 or V3
R2	Full range of motion achieved when muscle is at rest and tested at V1 velocity

Management

It important to consider the triggers of spasticity when planning interventions. There are a number of stimuli which can exacerbate spasticity, some may need multidisciplinary input alongside implementing physiotherapy management. These include:

• Skin: pressure ulcers, ingrown toe nails
• Bladder or bowel: constipation, full bladder
• Pain / discomfort: restrictive clothing, wheelchair straps, catheter straps
• Seating / positioning
• Fatigue
• Infections: UTI, pneumonia, skin
• Stress
• Disease progression
• Menstruation
• Other medical condistions (e.g. kidney stones)
• Ill fitting orthotics

Physiotherapy

Spasticity is one of the components of the UMN syndrome but should not be considered in isolation when it comes to management strategies. It is essential that management targets identified impairments to function and is always patient focused rather than aimed at reducing the degree of spasticity Planned interventions should consider a balance between movement and positioning. This includes:

• Standing
  • Standing frame
  • Treadmill training (body-weight supported if needed)
  • Tilt table
• Active exercises
• Passive movements
• Functional electrical stimulation
• 24 hour positioning management
• Splinting and the use of orthotics
• Stretches

Considerations for managing spasticity :

• Client care:
  • Preventing or treating contractures
  • Monitoring skin to preventing pressure areas
  • Positioning of trunk, head and limbs in supine and in chair/ wheel chair
    • If possible, standing promotes anti-gravity muscle activity in the trunk and lower limbs. It also maintains or improves soft tissue and joint flexibility and modulation of the neural component of spasticity.
  • Orthotics to maintain / improve available range
• Movement improvement:
  • The unmasking of voluntary movements previously covered by significant spasticity in cases of incomplete lesions
  • Accelerating the “spontaneous” recovery process
  • Modifying the “immature” motor pattern
  • Using new recovery techniques to promote guided neuroplasticity, e.g. robotic rehabilitation
  • New functional pattern in moving and walking.
• ADL’s and transfers
  • Getting around
  • Putting on clothes
  • Personal hygiene
  • Driving, etc.
  • Occupation
• Quality of life
  • Independent living
  • social and professional reintegration
  • Task specific activities can have a positive psychological effect

Patients should be educated on maintaining a daily stretching and range of motion program. In addition to the patient, the family and caregivers should be educated about proper positioning, daily skin inspection, an adequate and regular bowel/bladder regimen, avoiding noxious stimuli, and identifying signs of infection and pain.

Pharmacological

Some individuals may benefit from medications to manage spasticity. These can be generalised, focal or intrathecal interventions.

If the spasticity is widespread then systemic medication is used. This includes:

• Dantrolene (Dantrium)
• Baclofen (Lioresal and others)
• Tizanidine (Zanaflex)
• Diazepam (Vallium)
• Benzodiazepines
• Gabapentin
• Pregabalin
• Canabinoids

If the spasticity is locallised then local medication is used. This includes:

• Boutulinum Toxin (Botox)
• Regional Nerve Block

Intrathecal medications can also be used. These include:

• Baclofen
• Phenol

Medication
Baclofen	The most common systemic agent. Baclofen acts on the receptors of excitatory nerve terminals, in particular the ‘GABA B G-Protein receptor. Once the baclofen has attached to this G-Protein on the pre-synaptic terminal, potassium channels open while calcium channels close, hyperpolarising the cell. The inability for calcium to enter the cell means the release of glutamate, an excitatory neurotransmitter, is prohibited.
Tizanidine	Follows the same mechanism as Baclofen, however attaches to the a2 adrenoreceptor on the pre-synaptic cell membrane.
Botulinum Toxin (Botox)	Injected locally into muscle. Prevents the excytotic release of acetylcholine at the level of the neuromuscular junction which further prevents release of calcium from the sarcoplasmic reticulum which leads to excitation-contraction coupling.
Diazepam (Vallium)	Less common. Increases the effect of GABA, an inhibitory neurotransmitter, that is released from inhibitory interneurons which decreases the excitability within the post-synaptic nerve terminal.
Dantrolene	Provided orally. Blocks the release of calcium from the sarcoplasmic reticulum within the muscle which prevents excitation-contraction coupling.

Considerations of medication:

• Optimise timings of sessions alongside medications
• Exposing weakness in limbs of trunk
• Monitor effects and when dose may need adjusting
• Side effects, such as fatigue

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Definition

Amputation is defined as surgical removal or loss of body part such as arms or limbs in part or full.

Prevalence

One million limb amputations are reported globally each year. And as of 2017, 57.7 million people across the globe have been living with traumatic amputation. Approximately 185,000 amputations occur in United States each year according to the amputee coalition. And also, as of April 2021, United states has over 2 million Americans living with amputation, and another 28 million at a risk of surgical amputation due to undelaying causes.

Data from Stanford Healthcare shows 49% rise in total number of amputations during the time of COVID-19 pandemic, during March 2020 to February 2021.

Causes of Amputations

There are several conditions that can lead to amputation.

• Severe infection with extensive tissue damage
• Gangrene
• Trauma resulting from accident or injury, such as crush or blast wound
• Congenital/ Paediatric limb deficiency undergoing conversion amputation
• Congenital deformities of digits or limbs
• Congenital extra digits or limbs
• Necrosis or Necrotizing Fasciitis
• Cellulitis
• Peripheral Arterial Disease
• Frostbite
• Malignant/ cancerous tumor in bone or muscle of the limb e.g. Osteosarcoma
• Conditions that affect blood flow for example Diabetes

Levels of Amputation

Upper Limb 

• Forequarter
• Shoulder Disarticulation (SD)
• Transhumeral (Above Elbow AE)
• Elbow Disarticulation (ED)
• Transradial (Below Elbow BE)
• Hand/ Wrist Disarticulation
• Transcarpal (Partial Hand PH)
• Transmetacarpal

Lower Limb

• Hemicorporectomy
• Hemipelvectomy/ Hindquarter amputation
• Hip Disarticulation
• Short transfemoral(above knee)
• Transfemoral (above Knee)
• Long transfemoral (above knee)
• Knee Disarticulation
• Short transtibial (below knee)
• Transtibial (below knee)
• Long transtibial (below knee)
• Ankle Disarticulation (Symes)
• Tansmetatarsal
• Partial Foot/ray resection
• Toe disarticulation
• Partial Toe

Pre-Surgical Evaluation

1. General system review- Cardiovascular & Respiratory
2. Nutritional status
3. Diabetes Control if appropriate
4. Bowel & Bladder Function
5. Past medical history
6. Social history
7. Pre-morbid mobility
8. Strength & Condition of Healthy limb
9. Psychological assessment to access emotion impact of amputation
10. Home & work place assessment to make sure, everything is in accordance for patients maximal self reliance
11. Explanation of post-operative regimen.

Surgery for Amputation

Anesthesia is the first step to any surgery. During amputation, choice of anesthesia depends on the type of amputation, described above on levels of amputation. Two option of anesthesia for amputation are general anesthesia or epidural anesthesia.

While performing amputation, special care is to be taken to make sure the procedure does not hamper the functioning of remaining limb. It is vital to condition, shorten & smoothen the remaining bone, so there is a healthy stump that in future can take the load of a prosthetic limb and reduce complication risk.

Muscle is sutured to the bone at the distal residual bone so maximal strength of the remaining limb can be retained. This procedure is known as myodesis.

Distal stabilization of the muscles is always recommended, allowing for effective muscle contraction and reduced atrophy. This in turn allows for a greater functional use of the stump and maintains soft tissue coverage of the remnant bone. As the procedure for amputation is completed, the wound us sealed by performing myoplasty: suture to opposite muscle in the residual limb to to each other and to the periosteum or to the distal end of the cut bone for weight bearing purposes; and is covered with a bandage. A drainage tube might be placed to drain all excess fluid. Hence, every possible measure is taken to reduce risk of infection.

Ideal Stump

1. Skin flaps: skin should be mobile, sensation intact, no scars
2. Muscles are divided 3 to 5 cm distal to the level of bone resection
3. Nerves are gently pulled and cut cleanly, so that they retract well proximal to the bone level to reduce the complication of neuroma

Stump care

• For hygiene and skin care see handout on amputations
• A hip flexion contracture may develop because of elevation to reduce swelling
• Stump bandaging is done to ‘cone’ the stump, thereby preventing oedema, which occurs because there is no muscle pump and the stump hangs
• Swelling must be prevented to allow proper attachment of the prosthesis, and the prevention of pressure sores
• The stump sock is put on first, then the prosthesis
• The prosthesis must be cleaned and maintained (children who are still growing, grow out of their prostheses)

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Complications of Amputation

• Edema & Swelling
• Wounds Infection
• Pain (phantom limb)
• Muscle weakness
• Muscle Tightness & contractures
• Joint Instability
• Autonomic dysfunction
• for more detailed information on post-operative complications following an amputation.

Post Surgical Evaluation

• General system review- Cardiovascular & Respiratory
• Nutritional status
• Diabetes Control if appropriate
• Bowel & Bladder Function
• Strength & Condition of Healthy limb
• Psychological assessment to access emotion impact of amputation
• Signs of Infection
• Type of pain(Incisional, phantom, other)
• Level of Pain(VAS 1 – 10)
• Functional status (Bed mobility, transfers, sitting, standing, walking, balance)
• Strength and/or pain of the un-amputated limb

Post-operative Care

• Maintain function in the remaining leg and stump to maintain peripheral circulation
• Maintain respiratory function (important with smokers and those patients under general anaesthesia)
• Prepare for mobility rehabilitation

Pain in Amputation

Residual Limb pain and phantom limb pain are the two essential types of pain, post amputation.

Residual Limb Pain

Residual limb pain has three main cause:

1. Very likely post operative pain
2. Peripheral nerve neuroma formation at the end of cut peripheral nerve
3. Prosthetic pain caused by ill fitting prosthesis

Phantom Limb Pain

Phantom limb pain is defined as “pain that is localized in the region of the removed body part.” Cause of Phantom limb pain is not fully understood , but it is distressing and has a significant impact on patients life.

Psychological Implications of Amputation

• Loss of limb has a huge psychological impact on ones mental health, as if the person has lost a loved one. It is difficult to cope with loss of sensation and function from the amputated limb. It also changes your (patients) and other peoples perception of your (patients) body image, which can lead to depression and anxiety as negative thoughts are very common.
• Psychological well being of the patient is vital to a good rehabilitation process. Hence, it is the duty of a physiotherapist/ physical therapist to acknowledge patients concerns with good knowledge of natural grieving process.^[1][2]

Fall Risk Post Amputation

• Patient undergone amputation is at a higher risk of fall especially when they try to get up from the bed or chair and they have forgotten about the absence of the limb. These falls can cause injury to the surgical site, leading to prolonged healing.^[1]
• To deal with the risk of fall, it is mandatory to place a walker besides the patient. This reminds the patient to use assistance for transfers.

Goals of Post-operative Physiotherapy Management

1. Patient education on amputation and rehabilitation post amputation.
2. Maintenance of respiratory & cardiovascular status both pre- & post- surgery
3. Proper positioning of amputated limb to maintain the limb in right anatomical position.^[10]
4. Maintaining/improving strength of unamputated limb
5. Residual Limb care
6. Balance training
7. Transfer Training
8. Mobility Training
9. Prosthetic training

Post-operative Physiotherapy Management

1. Start with respiratory maintenance with pain control for amputated limb. Also start strengthening the unamputated limb to maintain its strength.
2. Teach the patient to wrap the residual limb correctly.
3. Prevent hip flexion contracture by avoiding prolonged sitting & guiding patient to spend more time in prone position.
4. For proper positioning avoid side lying position, maintain the residual limb in extension at hip & knee.
5. Start balance training
6. Procced to mobility training initially with a walker followed by crutches.
7. Start strengthening of the residual limb before proceeding to prosthetic training

Pre- Prosthetic Evaluation

1. Social History of patient
2. Financial status
3. Cause of amputation
4. Cardiopulmonary status examination
5. Scar healing assessment
6. Sensory assessment
7. Residual limb length & shape
8. Emotional status of patient for acceptance of amputation and body image
9. Vascularity (pulse, color of limb, temperature, edema/swelling, pain & tropic changes
10. Range of motion
11. Muscle strength
12. Pain (residual & phantom)
13. Functional status ( transfers, mobility, activities of daily living)

Please find below links to more detailed pages on the management of amputees

• Pain Management
• Pre-Fitting Management of the Patient with a Lower Limb Amputation
• Post-fitting Management
• Prosthetic Rehab
• High level Rehab
• Clinical Guidelines: Mental Health Amputees

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More to do with patients with amputation

1. Berger’s Exercises

• Stimulates collateral blood flow in the patient’s leg
• It is performed for 20 min.
• The leg is elevated until the toes go white, then lowered, then level
• Repeat 2-3 times to improve collateral circulation

2. Connective Tissue Massage

3. Dynamic Stump Exercises

4. Balance and Gait Retraining

• Improve static and dynamic balance
• Use parallel bars, walking frame then Crutches (in that order)
• Therapist stands on the amputation side, using a belt around the patient’s waist to support
• Rest if the patient feels tired

5. Short Wave Diathermy (SWD)

Through the pelvis to warm the arteries (contraindicated in patients with arterial insufficiency because the warmth leads to increased metabolism, causing a greater demand for nutrients, which are not available)

Walking Again Post Amputation/ Mobility Aids

• The choice of mobility aids depends on the level of fitness, strength, balance skills of the individual:
  • Walking frame
  • Axillary crutches
  • Elbow crutches
  • Walking stick 
• For bilateral lower limb amputees a wheelchair is often indicated (high energy expenditure during gait with prostheses)

Prosthetic Training

Prosthetic training is vital for smooth and energy efficient living, while performing all of daily living activities.

Below is step by step guidance to prosthetic training:

• Accepting weight of the
• body on each leg is vital to prosthetic training
• work on strengthening unamputated limb along with residual limb
• Teach the patient to balance on both legs and than on one leg
• Teach walking with prosthetic first with use of walker followed by crutches and stick
• Further proceed to independent prosthetic training.
• Teach the patient to adapt to environmental demands while walking

Introduction

The loss of a lower limb has severe implications for a person’s mobility, and ability to perform activities of daily living . This negatively impacts their participation and integration into society. The ultimate goal of rehabilitation after limb loss is to ambulate successfully with the use of a prosthesis and to return to a high level of social reintegration. Prosthetic rehabilitation is a complex task that ideally requires input from a transdisciplinary rehabilitation team. However, most often physiotherapists are in charge of the physical rehabilitation process.

Overview of the Rehabilitation Process

The rehabilitation process of the lower limb amputee consists of nine phases, namely:

1. Pre-operative
2. Amputation surgery
3. Acute post-surgical
4. Pre-prosthetic
5. Prosthetic prescription
6. Prosthetic training
7. Community integration
8. Vocational rehabilitation and
9. Follow up.

Table 1: Phases of amputee rehabilitation: Modified from Esquenazi & Meier cited in Esquenazi.

Phase	Hallmark	Link
Pre-Operative	Assess body condition, patient education, surgical level discussion, postoperative prosthetic plans	Assessment of the amputee
Amputation Surgery/Reconstruction	Length, myoplastic closure, soft tissue coverage, nerve, handling, rigid dressing
Acute Post-Surgical	Wound healing, pain control, proximal body motion, emotional support
Pre-Prosthetic	Shaping, shrinking, increase muscle strength, restore patient locus of control	Post-fitting management of the amputee
Prosthetic Prescription	Team consensus on prosthetic prescription and fabrication	Prosthetics
Prosthetic Training	Increase prosthetic wearing and functional utilisation	Gait in prosthetic rehabilitation
Community Integration	Resumption of roles in family and community activities. Emotional equilibrium and healthy coping strategies. Recreational activities.
Vocational Rehabilitation	Assess and plan vocational activities for the future. May need further education, training or job modification.
Follow-up	Life-long prosthetic, functional, medical assessment and emotional support

Throughout all of these phases, a rehabilitation treatment plan is utilised to guide the care of an individual who has undergone an amputation. The treatment plan is based on an evaluation by all specialties involved in the rehabilitation process and acts as a guide for all team members to address goals important to the patient and family. The level of rehabilitation intervention is contemplated from the date of admission to the hospital and determined after the amputation surgery and prior to discharge from the hospital. The rehabilitative process includes:

• Ongoing medical assessment of impairments, and
• Therapy interventions to address disabilities or activity limitation

Below we define this more detailed team-focused rehabilitation process in 8 phases:

	Patient Journey	The Team Management	Physiotherapy Management
1.	Pre-operative	Subjective assessment – History of present condition; Past medical history; Drug history; Social History.Objective assessment – Range of movement (ROM); Muscle power; Limb for amputation; Pulses; Skin integrity.Collaborating all findings to decide on the most appropriate level of amputation for the individual and, if they are likely to become a limb wearer, which level of amputation would be most appropriate.	The focus is on the objective assessment looking at ROM and muscle power. Using assessment findings, knowledge of prosthetic componentry and gait patterns, provide a clinically reasoned recommended level of amputation to the consultant. Provide patient with appropriate exercises to aid post-amputation mobility.
2.	Amputation surgery/reconstruction	Amputation surgery and reconstruction is the responsibility of the surgeon.
3.	Acute post-operative	Medical care; Wound care; Discharge planning; Rigid dressing used rigid material such as plaster of Paris applied immediately after surgery and kept in place for 5-7 days (only for below knee and below elbow).	Post-operative chest physiotherapy. Transfer practice and specific exercises to improve strength; increase exercise tolerance; maintain ROM.
4.	Pre-prosthetic rehabilitation	Monitoring patient progress; counselling if required; Patient goals; deciding on prosthetic prescription with the whole team and all appropriate information [including how the patient has managed with Early walking aids (EWA) and patient goals ]	Early walking aids (EWA) can be used to help decide on a patient’s suitability for a prosthetic limb.Exercise therapy to prepare the limb for a prosthesis.Liaison with the whole team regarding pre-fitting management of the amputee.
5.	Prosthetic prescription	Casting and measuring – Prosthetists cast and measure a patient’s residuum.Fitting of the prosthesis –  Prosthetists fit the primary prosthesis to the patient, ensuring the alignment and length are correct in standing and walking.	The physiotherapist may be needed to assist in the cast appointment, to ensure a neutral alignment of the pelvis is obtained.Physiotherapy provides intervention on mobility guidance, static balance and weight-bearing.
6.	Prosthetic Training	Prosthetists will help problem solve and adjust prosthesis as required.	The physiotherapist takes a lead role at this stage. Beginning with educating the patient about donning and doffing the prosthesis, skin integrity and weight-bearing areas on their residuum. A gait rehabilitation programme can then commence.
7	Discharge Management	The occupational therapists take a large role in this phase to aid the resumption of roles in family, recreational and community activities. They will also assist with vocational rehabilitation by assessing and planning vocational activities for the future. May need further education, training or job modification. Counsellors will be involved with emotional equilibrium and healthy coping strategies.	The physiotherapist should ensure that they include education for ongoing management, strategies for coping and training for resuming functional activities.
8.	Follow up	Patient will be reviewed regularly by the consultant the physiotherapist, and the prosthetic team.Assessing the individual is still suitable for prosthetic use, the prosthetic prescription is still the most appropriate option for the patient and any required changes are made to the prosthesis as the patient changes.	The consultant and/or prosthetist may ask for physiotherapy input. For example, if the patient is having a change of prescription, their goals have changed, their mobility has decreased/increased. The physiotherapist may be required to re-commence a gait rehabilitation programme with the patient or advice only may be required.

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Psychological Assessments or Psychological Tests are verbal or written tests formed to evaluate a person’s behaviour. Many types of Psychological tests help people understand various dynamics of the human being. It helps us understand why someone is good at something, while the other is good at another. However, Humans are complex beings which can’t be defined and classified under certain branches. The subjective nature of humans and individual differences has quite often raised criticism in psychological testing.

The classification of the types of psychological tests is as follows:

• As per the nature of psychological tests in terms of standardized and non-testing method of testing
• As per the functions of psychological tests such as intelligence tests, personality tests, interest inventories, aptitude tests, etc.

Characteristics of Psychological Tests

Here are the key characteristics of Psychological Tests:

• Reliability: The psychological assessment/test must produce the same result no matter when it’s taken.
• Validity: The psychological test must measure what it’s been created to assess.
• Objectivity: The assessment must be free from any personal bias for its scoring, interpretation of scoring or administration.
• Standardization: The test must be standardized in terms of its place, material and time for the assessment as well as its environment.

Types of Psychological Tests

Now that you know about their origins, let’s explore the top and most popular psychological tests.

Here are the major nine types of Psychological tests:

1. Personality Tests
2. Achievement Tests
3. Attitude Tests
4. Aptitude Tests
5. Emotional Intelligence Tests
6. Intelligence Tests
7. Neuropsychological Tests
8. Projective Tests
9. Observation (Direct) Tests

Types of Intelligence Tests

The pioneer of Intelligence Tests, Alfred Binet was the first one to construct the IQ test for assessing French students to identify which students need special assistance. He soon realized that a few students could solve much more advanced questions which an average student couldn’t. He then realized that various factors play a role in assessing the intelligence of a person which makes the term multi-dimensional. He then constructed the Binet-Simon test which was revised and given the name Stanford-Binet test which became the standard intelligence test in the U.S. Soon, many intelligence tests were formed for different groups of people. Psychologists started intervening the possible variables and Psychology tests like Wechsler Intelligence Scales, Raven’s Progressive Matrices, etc. 

Here are the major types of intelligence tests:

• Wechsler Individual Achievement Test
• Woodcock Johnson III Tests of Cognitive Disabilities
• Wechsler Adult Intelligence Scale
• Stanford-Binet Intelligence Scale
• Peabody Individual Achievement Test
• Universal Nonverbal Intelligence
• Differential Ability Scales

Personality Tests

Earlier, Phrenology (the measurement of the bumps on the skulls) was used to assess someone’s personality. A personality test evaluates our behaviours, emotions, behavioural and environmental traits, attitudes and even clinical disturbances in people. Each personality test is used to measure a certain variable or compare two variables. For example, adolescent emotional problems or psychopathologies are screened using The Minnesota Multiphasic Personality Inventory (MMPI-A). There are various versions of the MMPI depending on the sample type you want to test. 

Another unique type of personality test is the projective assessment. Very commonly used Projective assessments are the Thematic Apperception Test and the Rorschach Inkblot test. These projective psychological tests are formed to test the response of a person to a certain stimulus which elicits different hidden emotions, underlying thoughts or beliefs using pictures.

Here are the types of personality tests in psychology:

• Objective Tests of Personality: MMPI
• Projective Tests of Personality: Rorschach Inkblot Test ,The Thematic Apperception Test

Aptitude Tests

As mentioned above, humans have certain abilities and specialities. A person who has a creative bent of mind might not necessarily have a calculative brain. Such different abilities and interests are tested using aptitude tests. It is used to predict the future scope of a person or tests whether a person possesses a certain skill set. However, speaking of human complexities again, humans are incomprehensive and unpredictable in their ways which always leaves some room for ambiguity. Various aptitude tests are given to students and employees. Many capability tests also come under aptitude tests. (For eg.: Assessment of Edward who wants to become a police officer. For such an alert, courageous and risk-taking job, Edward must possess certain skills and abilities for being selected.) Some common forms of aptitude tests used are the Graduate Management Admission Test (GMAT), Graduate Record Examination (GRE), Scholastic Assessment Test (SAT), amongst others.

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Speed and Power Tests

Another type of Psychological tests on our list is the speed and power tests. It is a test where the performance is being measured based primarily upon the speed with which one works. The example can be tests of clerical ability. The other alternative can be where the test is difficult and the applicant or the person is given as much time as he/she wants. This type of tests where the person’s score is based exclusively upon his/her ability to answer the question correctly irrespective of the time he/she has taken is known as power test. An example can be of tests like Tweezers Dexterity Test etc. 

Emotional Intelligence Test

Have you ever felt like you impulsively shout at someone or cry without any reason? These emotions of anger and sadness is what we need to control and monitor. We need to identify our emotions and regulate them without being influenced by others. An Emotional Intelligence test taps various emotions through situations presented to the test-taker. An emotional intelligence  test requires a person’s honesty in it to accurately evaluate a person’s EQ [Emotional Quotient] and suggest ways to improve it. It is often noted that people who have higher EQ are much more content and successful than people otherwise. Even though emotional intelligence can overlap with other aspects like personality or genetic compositions, Emotional Intelligence of a person tends to fluctuate or change. It often requires constant consciousness in your actions and evaluation of its consequences. 

“When dealing with people, remember you are not dealing with creatures of logic, but with creatures of emotion.” – Dale Carnegie

Neuropsychological Tests

Now scroll up to the beginning of the blog where we mentioned different perceptions. These perceptions are due to the different neurological structures and pathways our brain has. These tests are designed to measure the cognitive workings of a person. How would you test if you have a strong or a weak memory? Neuropsychological tests are the most essential form among the many types of psychological tests used for assessing diseases like Alzheimer’s, Brain injury, Emotional disorders, such as depression or anxiety. It is important for doctors to know the core of the problem to cure it. Neurological tests assess factors like Memory, Language, Executive functioning, Dementia, Visuospatial Function, etc.

Individual and Group Tests

There are a number of tests which are meant to be performed individually. Such tests are called individual tests and these tests are preferred for vocational guidance and counselling and for clinical and diagnostic work with emotionally disturbed persons. As individual tests are more costly, therefore they are less used in the industry than the group tests. An example of an individual psychological test can be the Stanford -Binet intelligence scale. On the contrary, some tests are usually designed for a purpose so that they can be administered to a large number of people in the industry. The examples of group tests can be Purdue Vocational Achievement Tests, the Adaptability Test and the Wonderlic Personnel Test. 

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Essay and Objectives Tests

The essay tests are probably one of the oldest methods of psychological tests that are created to check the candidate’s ability to organise and articulate his or her thoughts clearly and of course logically. It is Lord Macaulay who has been credited with introducing this concept for the Indian Administrative Services or IAS. On the other hand, the objective test has one correct answer and does not require or ask for any sort of long extensive answers/explanation from the candidates. These tests are generally used to check the mental ability or mental power of the candidate and reasoning and clarity of the concepts above all. 

Uses of Psychological Testing

Psychological Tests are mainly used to analyse the mental abilities and attributes of an individual, including personality, achievement, ability and neurological functioning. Here are the central and most important uses of Psychological Testing:

• Detection of Specific Behavior
• Psychological Diagnosis
• Tools in Academic Placements
• Screening Job Candidates
• Individual Differences
• Research
• To Promote Self-awareness and Understanding
• Psychometrics/Career Assessment Tests
• Organizational Development

History of Psychological Testing

Originally created by Francis Baton as a group of tests, Psychological testing methods can be traced way back to 2200 B.C in China when an emperor tested his officials to know whether they were suitable for his office. Since then many Chinese dynasties have seen such tests unfold into more formal ones with various levels. These tests created an impression in the world and soon every country started following them. Fast forward to the time when the whole world was struck with World War I, this era served as a critical crunch in the psychological world. Many types of psychological tests were designed to evaluate soldiers for the army and to filter soldiers who were suffering from ‘shellshock’ or PTSD. Such intense screenings might come off as archaic in today’s world, but it was a landmark in psychology because it gave rise to the World’s first Personality Test.

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Functional assessments have become an integral part of the comprehensive rehabilitation medicine evaluation. Descriptions of improvements in function have been consistently performed since rehabilitation medicine developed after World War II. Unfortunately, previously utilized methods lacked the consistency required to study rehabilitation outcomes accurately.

Functional assessment measures an individual’s level of function and ability to perform specific tasks on a safe and dependable basis over a defined period. A detailed assessment should include a pertinent clinical history; a neurologic and musculoskeletal evaluation, a physical effort determination, and a comprehensive evaluation of behaviors that might impact physical performance. Assessments must be valid, reliable, and reproducible. They can be self-administered questionnaires or clinician administered.

From a research standpoint, functional assessments provide supporting evidence to develop, improve and attest to different evidence-based treatments. In the clinical setting, these instruments are commonly used to set rehabilitation goals, to develop specific therapeutic interventions and to monitor clinical changes.

In 2014, functional assessments took a different direction when the Improving Medicare Post-Acute Care Transformation Act of 2014 (the IMPACT Act) was signed into law seeking to connect findings on the baseline assessment to functional outcomes. This required that Long-Term Care Hospitals (LTCHs), Skilled Nursing Facilities (SNFs), Home Health Agencies (HHAs) and Inpatient Rehabilitation Facilities (IRFs) to report and submit standardized patient assessment data, including quality measures and standardized patient assessment data elements. The collection of this information permitted the exchange of information among providers on specific functional domains that included functional status, cognitive function, and mental status among some. The final goal intended to enhanced rehabilitation outcomes through share decision making, care coordination and improved discharge planning.

Relevance To Clinical Practice

The scope of practice in Rehabilitation Medicine is wide and includes an array of conditions such as neurological (stroke, TBI, neurodegenerative), musculoskeletal (joint pain, tendinopathies, ligamentous injuries, balance dysfunction) pain syndromes, medical (deconditioning, cardiopulmonary), rheumatologic (Rheumatoid Arthritis, Osteoarthritis, Connective Tissue Disorders), among others.

Commonly used assessments include:

Activities of daily living (Table 1A) measures the performance of basic functional skills required to care for oneself independently. They measure basic daily activities (eating, grooming, bathing, dressing, continence) mobility (gait, transfers) and cognition. Examples include: 

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• Barthel Index
• Functional Independence Measure (FIM)
• Functional Independence Measure for Children (WeeFIM)
• GG Functional Abilities and Goal 
• specific Functional Scale
• Canadian Occupational Performance Measure
• Lawton’s Instrumental Activities of Daily Living among others
• WHO International Classification of Functioning, Disability, and Health (ICF)
• International Classification of Functioning, Disability and Health for Children and Youth (ICF-CY)

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An exercise tolerance test (also called a stress test) shows how well your heart handles physical activity. While exercising on a treadmill or stationary bike, a healthcare provider will measure your heart rate, blood pressure, and breathing while monitoring an electrocardiogram (ECG), a test that records your heart’s electrical signals.

Exercise tolerance tests are generally used to detect certain heart conditions or determine safe levels of exercise for people with existing heart conditions.

This article provides an overview on what an exercise tolerance test entails and how to understand the test’s results.

Purpose of an Exercise Tolerance Test

Exercise tolerance tests evaluate how hard your heart works when it’s under the stress of physical activity.

When we exercise, our hearts pump harder and faster to deliver blood and oxygen throughout the body.¹ It’s often easier to detect certain heart conditions while your heart is working hard, since the heart may appear to be working normally while at rest.

After hooking a patient up to a blood pressure cuff and ECG during exercise on a treadmill or stationary bike, healthcare providers can monitor your:

• Heart rate
• Blood pressure
• Breathing
• Level of exertion

Not everyone needs an exercise tolerance test. They’re mainly used to detect a heart condition or monitor a preexisting heart condition. For example, a healthcare provider may recommend this test:

• If you have symptoms like chest pain or shortness of breath that could indicate a heart condition
• If you have an irregular heartbeat
• To determine a safe exercise plan for patients recovering from heart surgery
• To see if treatments you’re receiving for heart disease are working well

Recap

An exercise tolerance test can demonstrate your heart’s ability to endure physical exercise. This in-office exam is often used to help detect heart conditions and determine appropriate treatments or exercise plans for existing heart patients.⁴

Limitations of an Exercise Tolerance Test

As with any medical test, exercise tolerance tests have some limitations. For example:

• Exercise tolerance tests can help detect an artery blockage in coronary artery disease, but they can’t predict things like how much of the artery has thickened or if and when a person may have a heart attack.
• Exercise tolerance test results aren’t always 100% accurate and can vary based on equipment used, experience of the healthcare professional conducting the test, and other factors. Some people may get a false-positive result, indicating a heart condition when there is none.

With this in mind, your healthcare provider will typically use the results of an exercise tolerance test along with other information, like your symptoms, medical history, and results of other tests, to make a determination or diagnosis.

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Recommendations for Exercise Testing

The U.S. Preventive Services Task Force recommends against routine exercise tolerance testing for people who don’t have any heart symptoms and are at low risk for developing heart disease. This is because the risk of false-positive findings can result in unnecessary further testing, treatment, or worry.

Risks of an Exercise Tolerance Test

An exercise tolerance test is a relatively safe test.

That said, sometimes increasing your heart rate through exercise can cause symptoms like dizziness, chest pain, or nausea. A healthcare provider will closely monitor you throughout the test to reduce the chances of this happening and quickly treat any issues that may come up.

In addition, people with certain preexisting heart disease can experience complications like an irregular heartbeat or even a heart attack. To be safe, your healthcare provider would likely not recommend an exercise tolerance test if you have any of the following heart conditions:⁷

• Unstable angina (chest discomfort caused by poor blood flow)
• Uncontrolled arrhythmia (an irregular heartbeat)
• Pulmonary hypertension (a type of high blood pressure)
• Severe aortic stenosis (narrowing of the main artery)
• Congestive heart failure (a condition in which the heart doesn’t pump blood as well as it should)
• Pulmonary embolism (a clot in the arteries of a lung)
• Aortic dissection (a tear in the main artery)
• Another acute illness

Before the Test

There’s not much preparation needed for an exercise tolerance test. Your healthcare provider will give you a set of guidelines to follow, which may include these instructions:⁸

• Avoid eating a heavy meal or consuming anything other than water within a few hours of the test.
• Avoid consuming caffeine the day before the test.
• Wear loose-fitting clothing and comfortable walking shoes.
• Continue to take any usual medication unless otherwise advised by a healthcare provider.

During the Test

Exercise tolerance tests take place at a healthcare provider’s office or at a hospital and will be overseen by a medical professional. They are relatively quick and painless, minus any potential minor discomfort felt while exercising. The test itself should take about 15–20 minutes. 

Here’s what you can expect:⁹

1. Equipment used will include a treadmill or exercise bike, ECG machine connected to electrodes (wires), a blood pressure cuff, and a pulse oximeter to measure blood oxygen levels.
2. The small electrodes will be stuck to the skin of your chest or shoulders and attached to the ECG machine to measure your heartbeat and heart waves. In addition, the blood pressure cuff will be wrapped around your arm, and the pulse oximeter sensor will be clipped to your finger. 
3. You’ll then be asked to start exercising on the treadmill or bike at an easy pace. Gradually, the speed and incline are increased while the ECG monitors your body and heart’s reaction to the stress of exercise.
4. You may be asked to breathe into a mouthpiece that will measure how much air you’re breathing out.

Before, during, and after the test, your ECG, heart rate, blood pressure, and breathing will be monitored and recorded.

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After the Test

When the test is done, you’ll be able to sit or lie down for several minutes until your heart rate returns to its normal resting state. You may be offered a glass of water or towel while you cool down.

If your blood pressure substantially rose during the test, or if the healthcare provider noticed any other concerning symptoms, you may be monitored for a few additional minutes.

After getting clearance, you’ll be free to return home and await the test results, which usually come back within a couple of days.

Interpreting Results of an Exercise Tolerance Test

After the test is performed, your healthcare provider will look over the results. They’ll be evaluating factors like:¹⁰

• Your ECG reading
• Blood pressure changes
• Heartbeat changes
• Any symptoms reported or noted during the test
• Your estimated exercise capacity 

After summarizing these items, your provider will go over the results with you and offer a conclusion based on their interpretation. Possible results include:¹¹

• Positive (or abnormal): This means the test found potential evidence of a heart condition.
• Negative (or normal): This means the test didn’t find any evidence of a heart condition.
• Inconclusive or uninterpretable: This indicates the test was unable to determine if a heart condition is present. In this case, the equipment may have malfunctioned, or you were unable to complete the test.
• Goal achieved: If you took the test to help establish a safe exercise plan following a heart diagnosis or surgery, your healthcare provider might have set some fitness goals that you were able to meet.

Recap

Stress tests give your healthcare provider important information about the likelihood of a heart problem and to recommend next steps for you. Ultimately, your healthcare provider is the best source of information about interpreting your stress test results.

Follow-Up

Depending on the results, your healthcare provider may want to speak with you about further testing, alternative testing, or treatment options.

In some cases, more appointments or testing may be necessary to:

• Confirm your exercise tolerance test result
• Seek additional information after your exercise tolerance test result

Follow-up tests may include:

• MRI (magnetic resonance imaging) heart scan to evaluate the structure of your heart
• CT coronary scan to look at the arteries that supply blood to your heart
• Myocardial perfusion scan to provide a visual on how well the heart is pumping
• Nuclear stress test to measure the blood flow to your heart

Summary

An exercise tolerance test is one way to record your heart’s response to the stress of physical exercise. It measures the heart’s electrical activity, heart rate, blood pressure, and breathing while you’re on a treadmill or exercise bike.

Exercise tolerance tests may be used to diagnose certain heart conditions, monitor heart attack recovery, or form an appropriate fitness plan for heart disease or surgery patients. Your healthcare provider may recommend this test if you report symptoms of potential heart disease, such as chest pain or shortness of breath. This test generally isn’t recommended for people at low risk of developing heart disease.

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Key facts by WHO-

• Over 1 billion people live with some form of disability.
• The number of people with disability are dramatically increasing. This is due to demographic trends and increases in chronic health conditions, among other causes.
• Almost everyone is likely to experience some form of disability – temporary or permanent – at some point in life.
• People with disability are disproportionately affected during the COVID-19 pandemic.
• When people with disability access health care, they often experience stigma and discrimination, and receive poor quality services.
• There is an urgent need to scale up disability inclusion in all levels of the health system, particularly primary health care.

overview

A disability is any condition of the body or mind (impairment) that makes it more difficult for the person with the condition to do certain activities (activity limitation) and interact with the world around them (participation restrictions).

There are many types of disabilities, such as those that affect a person’s:

• Vision
• Movement
• Thinking
• Remembering
• Learning
• Communicating
• Hearing
• Mental health
• Social relationships

Although “people with disabilities” sometimes refers to a single population, this is actually a diverse group of people with a wide range of needs. Two people with the same type of disability can be affected in very different ways. Some disabilities may be hidden or not easy to see.

Disability is part of being human. Almost everyone will temporarily or permanently experience disability at some point in their life. Over 1 billion people – about 15% of the global population – currently experience disability, and this number is increasing due in part to population ageing and an increase in the prevalence of noncommunicable diseases. 

Disability results from the interaction between individuals with a health condition, such as cerebral palsy, Down syndrome and depression, with personal and environmental factors including negative attitudes, inaccessible transportation and public buildings, and limited social support.

A person’s environment has a huge effect on the experience and extent of disability. Inaccessible environments create barriers that often hinder the full and effective participation of persons with disabilities in society on an equal basis with others. Progress on improving social participation can be made by addressing these barriers and facilitating persons with disabilities in their day to day lives.

Disability can be:

• Related to conditions that are present at birth and may affect functions later in life, including cognition (memory, learning, and understanding), mobility (moving around in the environment), vision, hearing, behavior, and other areas. These conditions may be
  • Disorders in single genes (for example, Duchenne muscular dystrophy);
  • Disorders of chromosomes (for example, Down syndrome); and
  • The result of the mother’s exposure during pregnancy to infections (for example, rubella) or substances, such as alcohol or cigarettes.
• Associated with developmental conditions that become apparent during childhood (for example, autism spectrum disorder and attention-deficit/hyperactivity disorder or ADHD)
• Related to an injury (for example, traumatic brain injury or spinal cord injuryexternal icon).
• Associated with a longstanding condition (for example, diabetes), which can cause a disability such as vision loss, nerve damage, or limb loss.
• Progressive (for example, muscular dystrophy), static (for example, limb loss), or intermittent (for example, some forms of multiple sclerosisexternal icon).

Magnitude of disability –

About 15% of the world’s population lives with some form of disability, of whom 2-4% experience significant difficulties in functioning. The global disability prevalence is higher than previous WHO estimates, which date from the 1970s and suggested a figure of around 10%.

As per the country’s latest statistics, an estimated 2.1 percent of the Indian population has a disability; a humbling figure of over 26 million people. A person can face disability — intellectually and physically — at any point in their life from medical complications or life-altering events.

Epidemiology 

Epidemiology of Intellectual Disability and Comorbid Conditions Epidemiology is essentially the study of a disorder in a given population. Knowledge of the distribution of a disorder in a population can increase understanding of the causes and how best to manage it.

Management

Increasing evidence suggests that, as a group, people with disabilities experience poorer levels of health than the general population. By improving access to quality, affordable health care services, health outcomes for people with disabilities can be improved. Primary health-care services with the support of specialists can provide health services to people with disability. Health services should be focused for the following health conditions:   

Primary health conditions: A primary health condition is the possible starting point for impairment can lead to a wide range of impairments, including mobility, sensory, mental, and communication impairments.

Secondary conditions: Secondary conditions occur in addition to (and are related to) a primary health condition, and are both predictable and therefore preventable; such as depression is a common secondary condition in people with disabilities, osteoporosis is common in people with a spinal cord injury or cerebral palsy.

Co-morbid conditions occur in addition to (and are unrelated to) a primary health condition associated with disability. One study indicated that adults with developmental disabilities had a similar or greater rate of chronic health conditions such as high blood pressure, cardiovascular disease, and diabetes (due to increased physical inactivity) than people without disabilities.

Age-related conditions: The ageing process for some groups of people with disabilities begins earlier than usual and they may experience age related health conditions (such as osteoporosis, loss of strength and balance) more frequently.

Risk behaviours: People with disabilities have higher rates of engaging in risky behaviours such as smoking, alcohol conumption, poor diet and physical inactivity as compare to general population.

Violence :People with disabilities are at greater risk of violence than those without disabilities.

Unintentional injury: People with disabilities are at higher risk of unintentional injury from road traffic crashes, burns, falls, and accidents related to assistive devices.

Assistive technologies and assistive devices such as crutches, prostheses, wheelchairs, and tricycles in mobility impairments; hearing aids and cochlear implants for hearing impairments; ocular devices, talking books, and software for screen magnification and reading for people with visual impairments may be advised according to the user and the user’s environment.

Rehabilitation: It is an important aspect of management for people with disability. It involves combined and coordinated use of medical, social, educational, and vocational measures for training or retraining the individual to the highest possible level of functional ability.

Community-based rehabilitation (CBR) was initiated by WHO to enhance the quality of life for people with disabilities and their families; meet their basic needs; and ensure their inclusion and participation. CBR is implemented through the combined efforts of people with disabilities, their families and communities, and relevant government and non-government health, education, vocational, social and other services.  

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Role of Physiotherapy for disabled people

Disabled people spend their time in same position which affect their range of motion,when body is not mobile,that sedentary life style can affect the health and also weight of disabled people.With the help of physiotherapist the person with disability can stretch and strengthen muscles,this will help to give them more mobility and give positive impact on overall body.

• Decrease Pain: physiotherapist not only help the people to keep them fit but also help to reduce pain and stress.
• Physiotherapist prescribed mobility equipments to help disabled people stand and walk without help of other people like family members or friends.
• Physiotherapist asses, diagnose and provide exercise programs to strengthen the muscles.

For example

Strengthening exercises improve function, quality of life and reduce risk of injury.

Balance exercise helps in preventing falls

Relaxation exercise helps patient to calm mind and progressive muscle relaxation (PMR) relax muscle and reduce tension.

Flexibility exercise increase range of motion, decrease pain and stiffness.

      “During exercise endorphins release to energize mood, relieve stress and also boost self esteem”.

The Equality Act 2010 states “that disabled people should be treated equally, and protection from discrimination applies in many situations such as education, employment, exercise of public functions, goods, services, facilities and transport”.

Goals

• Education:

Should have the right to quality education at all levels of disabled people.

• Employment: 

Disabled people have the right to work in an open and accessible environment.

• Health care: 

They have right to highest standard of health care without discrimination.

Conclusion

People with disabilities have a higher rate of needs than non-disabled. The result indicates the importance of needs and wants of parents and patients should be respected so that the parents play an active role. There is a need to increase the rehabilitation center and staff to make them accessible for their needs in rural areas. Disabled people face problems in dealing with healthcare facilities, the barriers increase when a person is disabled and it can be decreased by giving them education and facilities which are easily accessible.

Occupational therapists ensure the people recovering from disability and promote physical and mental health through therapeutic use of everyday activities. They teach self care skills (cooking, eating, dressing) and enable them to return to their life.

On the other hand, Physiotherapists help the disabled people through exercise, manual therapy, electrotherapy, mobilization, manipulation for the management of pain and improve the range of motion.

legislation and act related to disablility

The Department administers the following three Acts:-

1. The Rights of Persons with Disabilities Act, 2016
2. The National Trust for the Welfare of Persons with Autism, Cerebral Palsy, Mental Retardation and Multiple Disabilities Act, 1999
3. Rehabilitation Council of India Act, 1992

The Rights of Persons with Disabilities (RPwD) Act, 2016

The RPwD Act, 2016 was enacted on 28.12.2016 which came into force from 19.04.2017. The salient features of the Act are:-

1. Responsibility has been cast upon the appropriate governments to take effective measures to ensure that the persons with disabilities enjoy their rights equally with others.
2. Disability has been defined based on an evolving and dynamic concept.
3. The Act covers the following specified disabilities:-

1. Physical Disability
   1. Locomotor Disability
      1. Leprosy Cured Person
      2. Cerebral Palsy
      3. Dwarfism
      4. Muscular Dystrophy
      5. Acid Attack Victims
   2. Visual Impairment
      1. Blindness
      2. Low Vission
   3. Hearing Impairment
      1. Deaf
      2. Hard of Hearing
   4. Speech and Language Disability
2. Intellectual Disability
   1.  Specific Learning Disabilities
   2. Autism Spectrum Disorder
3. Mental Behaviour (Mental Illness)
4. Disability caused due to-
   1. Chronic Neurological Conditions such as-
      1. Multiple Sclerosis
      2. Parkinson’s Disease
   2. Blood Disorder-
      1. Haemophilia
      2. Thalassemia
      3. Sickle Cell Disease
5. Multiple Disabilities

1. Additional benefits have been provided for persons with benchmark disabilities and those with high support needs.
2. Every child with benchmark disability between the age group of 6 and 18 years shall have the right to free education.
3. 5% reservation in seats in Government and Government aided higher educational institutions for persons with benchmark disabilities.
4. Stress has been given to ensure accessibility in public buildings (both Government and private) in a prescribed time-frame.
5. 4% reservation in Government jobs for certain persons or class of persons with benchmark disability.
6. The Act provides for grant of guardianship by District Court or any authority designated by the State Government under which there will be joint decision – making between the guardian and the persons with disabilities.
7. Broad based Central & State Advisory Boards on Disability to be set up as policy making bodies.
8. The Act provides for strengthening of the Office of Chief Commissioner of Persons with Disabilities and State Commissioners of Disabilities which will act as regulatory bodies and Grievance Redressal agencies and also monitor implementation of the Act. These Offices will be assisted by an Advisory Committee comprising of experts in various disabilities.
9. Creation of National and State Fund to provide financial support to the persons with disabilities.
10. The Act provides for penalties for offences committed against persons with disabilities.
11. Designated special Courts to handle cases concerning violation of rights of PwDs.

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THE NATIONAL POLICY FOR PERSONS WITH DISABILITIES, 2006

Recognizing that the Persons with Disabilities constitute a valuable human resource for the country and that a majority of such persons can lead a better quality of life if they have equal opportunities and effective access to rehabilitation measures, the Government, with a view to create an environment that provides such persons equal opportunities for protection of their rights and full participation in society, formulated and brought out the National Policy for Persons with Disabilities.

2. With focus on Prevention of Disabilities and Rehabilitation Measures, the Policy provides for the following:-

I.      Prevention of Disabilities

II.     Rehabilitation Measures

II A. Physical Rehabilitation Strategies :

• Early Detection and Intervention
• Counselling & Medical Rehabilitation
• Assistive Devices
• Development of Rehabilitation Professionals

II B. Education for Persons with Disabilities

II C. Economic Rehabilitation of Persons with Disabilities :

• Employment in Government Establishments
• Wage employment in Private Sector
• Self-employment

III.    Provisions for Women with Disabilities

IV.   Provisions for Children with Disabilities

V.    Barrier-free Environment

VI.   Issue of Disability Certificates

VII.  Social Security

VIII. Promotion of Non-Governmental Organizations (NGOs)

IX.   Collection of regular information on Persons with Disabilities

X.    Research

XI.   Sports, Recreation and Cultural life

XII.  Amendments to existing Acts dealing with the Persons with Disabilities

4. Accordingly, the principal areas of intervention under the Policy are :  Prevention, Early-detection and Intervention; Programmes of Rehabilitation; Human Resource Development; Education of Persons with Disabilities; Employment; Barrier free-environment; Social Protection; Research; Sports, Recreation and Cultural Activities.

5.       The following mechanism is in place for implementation of the National Policy:

        i. Department of Empowerment of Persons with Disabilities, Ministry of Social Justice & Empowerment is the nodal Department to coordinate all             matters relating to implementation of the Policy.

1.  The Central Coordination Committee, with stakeholder representation, coordinates matters relating to implementation of the National Policy. There is a similar Committee at the State level.
2. The Ministries of Home Affairs; Health & Family Welfare; Rural Development; Urban Development; Youth Affairs & Sports; Railways; Science & Technology; Statistics & Programme Implementation; Labour; Panchayati Raj and Women & Child Development and Departments of Elementary Education & Literacy, Secondary & Higher Education; Road Transport & Highways; Public Enterprises; Revenue; Information Technology and Personnel & Training are also identified for implementation of the policy.
3.  Panchayati Raj Institutions and Urban Local Bodies are associated in the functioning of the District Disability Rehabilitation Centres. They are required to play a crucial role in the implementation of the National Policy to address local level issues.
4. The Chief Commissioner for Persons with Disabilities at Central level and State Commissioners at the State level, play key role in implementation of the National Policy, apart from their respective statutory responsibilities.

UNITED NATIONS CONVENTION ON THE RIGHTS OF PERSONS WITH DISABILITIES (UNCRPD), 2006

The Convention was adopted by UN General Assembly on December 13, 2006 and opened for signing by the State Parties on March 30, 2007. Adoption of the Convention has really imparted empowerment to Persons with Disabilities across the globe to demand their rights and make State, private and civil society agencies accountable for enjoying their rights.

2. India is one of the few first countries which ratified the Convention. Consequent upon signing the Convention on March 30, 2007, India  ratified the Convention on 01.10.2007. The Convention has come into force from May 3, 2008. The Convention places the following three important obligations on each State Party: –

a) Implementation of the provisions of the Convention;

b) Harmonization of the country laws with the Convention and

c) Preparation of a Country Report.

3. Taking concrete measures for effective implementation of the Convention, all the concerned Central Ministries were requested to implement the provisions of the Convention as might be applicable to each of them. Similarly, all the Chief Ministers of States and Administrators of UTs were also requested to examine different provisions/obligations under the Convention as might relate to them and to take effective steps for their early implementation. The State Governments/UT Administrators were also asked to furnish a status report in this regard so that the same could be utilized towards preparing the Country Report. Rigorous monitoring and follow-up was being done in this regard so as to fulfill obligations of the Convention. India’s First Country Report was submitted to UN Committee on Rights of Persons in November, 2015.

Incheon Strategy

Incheon Strategy “To make the Right Real” for Persons with Disabilities in Asia and Pacific. The Ministers and representative of members and associate members of the United Nations Economic and Social Commission for Asia and the Pacific (ESCAP) assembled at the High Level Inter Governmental meeting on the final review of the implementation of the Asian and Pacific Decade of Disabled Persons, 2003-2012 held at Incheon, Korea from 29th October – 2 nd November, 2012 and adopted the Incheon Strategy “Make the Right Real” for Persons with Disabilities in Asia and the Pacific. The ESCAP in its 69th Session held from 25th April – 1 st May, 2013 passed the resolution endorsing the Ministerial declaration and Incheon Strategy.

2. The Incheon Strategy to make the Right Real for Persons with Disabilities in Asia and Pacific sets out the following 10 goals:-

1. Reduce Poverty and enhance work and employment prospects;
2. Promote participation in political processes and in decision making;
3. Enhance access to the physical environment, public transportation, knowledge, information and communication;
4. Strengthen social protection;
5. Expand early intervention and education of children with disabilities;
6. Ensure gender equality and women‟s empowerment;
7. Ensure disability-inclusive disaster risk reduction and management;
8. Improve the reliability and comparability of disability data;
9. Accelerate the ratification and implementation of the Convention on the Rights of Persons with Disabilities and harmonisation of National Legislation with the Convention; and x. Advance sub-regional, regional and inter-regional co-operation.

3. The Incheon Strategy spells out Action Plans at the regional, sub-regional and regional levels to achieve the above goals and also contains the core indicators for tracking the progress of implementation.

4.  In order to oversee implementation of the Incheon Strategy, the UNESCAP has constituted a Working Group on the Asian and Pacific Decade for Persons with Disabilities, 2013-2022. The Working Group consists for Government representatives from 15 member countries including India and 15 Civil Society Organisations in the Asia and Pacific Region. The First meeting of the Working Group was held at Incheon, Korea during 25-26 February, 2014. The Second Session of the Working Group was held in Delhi during 2-3 March, 2015 hosted by Department of Empowerment of Persons with Disabilities in association with UNESCAP Secretariat. Third and Fourth Session of the Working Group were held in Bangkok.

5. Subsequently, high-level inter-Governmental meeting was held on mid-point review of the Asia Pacific Decade for Persons with Disabilities in Beijing from 27^th November to 1^st December, 2017. After deliberations in the meeting, Beijing Declaration was adopted which outlines action plan for the State parties to achieve the targets of Incheon Strategy in the next five years.

Scheme

Research and Development

The Department of Empowerment of Persons with Disabilities has launched a new Central sector scheme on “Research on disability related technology, products and issues‟. In January, 2015 with the objective to promote research of service models and programmes on the basis of life cycle needs, holistic development of the individuals and their families and creating an enabling environment for the empowerment of the persons with disabilities and promote research in prevention and prevalence of disability and the application of science & technology for the development of indigenous, appropriate aids and appliances.

The scheme has 2 components

(i) Research and Development of assistive technology and product development devices; and

(ii) Scheme for study/research/survey/internship and periodical collection of data related to disability. The State Governments, the national institutes under the Department have been requested to submit their proposal in accordance with the scheme. Until 2017-18 the scheme was implemented as a standalone scheme of the Department. From the year 2018-19 it has become part of the umbrella scheme SIPDA.

2. The Department has developed guidelines for grant of financial assistance for R&D projects under SIPDA.

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