Medicurio

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INTRODUCTION-

Multiple sclerosis (MS) is a potentially disabling disease of the brain and spinal cord (central nervous system).

In MS, the immune system attacks the protective sheath (myelin) that covers nerve fibers and causes communication problems between your brain and the rest of your body. Eventually, the disease can cause permanent damage or deterioration of the nerves.

Signs and symptoms of MS vary widely and depend on the amount of nerve damage and which nerves are affected. Some people with severe MS may lose the ability to walk independently or at all, while others may experience long periods of remission without any new symptoms.

There’s no cure for multiple sclerosis. However, treatments can help speed recovery from attacks, modify the course of the disease and manage symptoms.

Multiple sclerosis is a chronic disease that affects the central nervous system, especially the brain, spinal cord, and optic nerves. This can lead to a wide range of symptoms throughout the body.

It is not possible to predict how multiple sclerosis (MS) will progress in any individual.

Some people have mild symptoms, such as blurred vision and numbness and tingling in the limbs. In severe cases, a person may experience paralysis, vision loss, and mobility problems. However, this is rare.

It is difficult to know precisely how many people have MS. According to the National Institute for Neurological Disorders and Stroke (NINDS), 250,000–350,000 people in the United States are living with MS.

The National Multiple Sclerosis Society estimate the number could be closer to 1 million.

However, new treatments are proving effective at slowing the disease.

What is MS?

Scientists do not know exactly what causes MS, but they believe it is an autoimmune disorder that affects the central nervous system (CNS). When a person has an autoimmune disease, the immune system attacks healthy tissue, just as it might attack a virus or bacteria.

In the case of MS, the immune system attacks the myelin sheath that surrounds and protects the nerve fibers, causing inflammation. Myelin also helps the nerves conduct electrical signals quickly and efficiently.

Multiple sclerosis means “scar tissue in multiple areas.”

When the myelin sheath disappears or sustains damage in multiple areas, it leaves a scar, or sclerosis. Doctors also call these areas plaques or lesions. They mainly affect:

• the brain stem
• the cerebellum, which coordinates movement and controls balance
• the spinal cord
• the optic nerves
• white matter in some regions of the brain

As more lesions develop, nerve fibers can break or become damaged. As a result, the electrical impulses from the brain do not flow smoothly to the target nerve. This means that the body cannot carry out certain functions.

Types of MS

There are four types of MS:

Clinically isolated syndrome (CIS): This is a single, first episode, with symptoms lasting at least 24 hours. If another episode occurs at a later date, a doctor will diagnose relapse-remitting MS.

Relapse-remitting MS (RRMS): This is the most common form, affecting around 85% of people with MS. RRMS involves episodes of new or increasing symptoms, followed by periods of remission, during which symptoms go away partially or totally.

Primary progressive MS (PPMS): Symptoms worsen progressively, without early relapses or remissions. Some people may experience times of stability and periods when symptoms worsen and then get better. Around 15% of people with MS have PPMS.

Secondary progressive MS (SPMS): At first, people will experience episodes of relapse and remission, but then the disease will start to progress steadily.

SYMPTOM-

Because MS affects the CNS, which controls all the actions in the body, symptoms can affect any part of the body.

The most common symptoms of MS are:

Muscle weakness: People may develop weak muscles due to lack of use or stimulation due to nerve damage.

Numbness and tingling: A pins and needles-type sensation is one of the earliest symptoms of MS that can affect the face, body, or arms and legs.

Lhermitte’s sign: A person may experience a sensation like an electric shock when they move their neck, known as Lhermitte’s sign.

Bladder problems: A person may have difficulty emptying their bladder or need to urinate frequently or suddenly (urge incontinence). Loss of bladder control is an early sign of MS.

Bowel problems: Constipation can cause fecal impaction, which can lead to bowel incontinence.

Fatigue: This can undermine a person’s ability to function at work or at home. Fatigue is one of the most common symptoms of MS.

Dizziness and vertigo: These are common problems, along with balance and coordination issues.

Sexual dysfunction: Both males and females may lose interest in sex.

Spasticity and muscle spasms: This is an early sign of MS. Damaged nerve fibers in the spinal cord and brain can cause painful muscle spasms, particularly in the legs.

Tremor: Some people with MS may experience involuntary quivering movements.

Vision problems: Some people may experience double or blurred vision, a partial or total loss of vision, or red-green color distortion. This usually affects one eye at a time. Inflammation of the optic nerve can result in pain when the eye moves. Vision problems are an early sign of MS.

Gait and mobility changes: MS can change the way people walk, because of muscle weakness and problems with balance, dizziness, and fatigue.

Emotional changes and depression: Demyelination and nerve-fiber damage in the brain can trigger emotional changes.

Learning and memory problems: These can make it difficult to concentrate, plan, learn, prioritize, and multitask.

Pain: Pain is a common symptom in MS. Neuropathic pain is directly due to MS. Other types of pain occur because of weakness or stiffness of muscles.

Less common symptoms include:

• headache
• hearing loss
• itching
• respiratory or breathing problems
• seizures
• speech disorders
• swallowing problems

There is also a higher risk of:

• urinary tract infections
• reduced activity and loss of mobility

These can impact a person’s work and social life.

In the later stages, people may experience changes in perception and thinking and sensitivity to heat.

MS affects individuals differently. For some, it starts with a subtle sensation, and their symptoms do not progress for months or years. Sometimes, symptoms worsen rapidly, within weeks or months.

A few people will only have mild symptoms, and others will experience significant changes that lead to disability. However, most people will experience times when symptoms worsen and then get better.

Lhermitte’s sign is a common symptom of MS that happens when a person moves their head.

When to see a doctor

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See a doctor if you experience any of the above symptoms for unknown reasons.

Disease course

Most people with MS have a relapsing-remitting disease course. They experience periods of new symptoms or relapses that develop over days or weeks and usually improve partially or completely. These relapses are followed by quiet periods of disease remission that can last months or even years.

Small increases in body temperature can temporarily worsen signs and symptoms of MS, but these aren’t considered true disease relapses.

At least 50% of those with relapsing-remitting MS eventually develop a steady progression of symptoms, with or without periods of remission, within 10 to 20 years from disease onset. This is known as secondary-progressive MS.

The worsening of symptoms usually includes problems with mobility and gait. The rate of disease progression varies greatly among people with secondary-progressive MS.

Some people with MS experience a gradual onset and steady progression of signs and symptoms without any relapses, known as primary-progressive MS

RISK FACTOR-

Scientists do not really know what causes MS, but risk factors include:

Age: Most people receive a diagnosis between the ages of 20 and 40 years.

Sex: Most forms of MS are twice as likely to affect women than men.

Genetic factors: Susceptibility may pass down in the genes, but scientists believe an environmental trigger is also necessary for MS to develop, even in people with specific genetic features.

Smoking: People who smoke appear to be more likely to develop MS. They tend to have more lesions and brain shrinkage than non-smokers.

Infections: Exposure to viruses, such as Epstein-Barr virus (EBV), or mononucleosis, may increase a person’s risk of developing MS, but research has not shown a definite link. Other viruses that may play a role include human herpes virus type 6 (HHV6) and mycoplasma pneumonia.

Vitamin D deficiency: MS is more common among people who have less exposure to bright sunlight, which is necessary for the body to create vitamin D. Some experts think that low levels of vitamin D may affect the way the immune system works.

Vitamin B12 deficiency: The body uses vitamin B when it produces myelin. A lack of this vitamin may increase the risk of neurological diseases, such as MS.

Previous theories have included exposure to canine distemper, physical trauma, or aspartame, an artificial sweetener, but there is no evidence to support these

There is probably no single trigger for MS, but multiple factors may contribute.

COMPLICATION-

People with multiple sclerosis may also develop:

• Muscle stiffness or spasms
• Paralysis, typically in the legs
• Problems with bladder, bowel or sexual function
• Mental changes, such as forgetfulness or mood swings
• Depression
• Epilepsy

DIAGNOSIS-

The doctor will carry out a physical and neurological examination, ask about symptoms, and consider the person’s medical history.

No single test can confirm a diagnosis, so a doctor will use several strategies when deciding whether a person meets the criteria for a diagnosis.

These include:

• MRI scans of the brain and spinal cord, which may reveal lesions
• spinal fluid analysis, which may identify antibodies that suggest a previous infection
• an evoked potential test, which measures electrical activity in response to stimuli

Other conditions have symptoms that are similar to those of MS, so a doctor may suggest other tests to assess for other possible causes.

If the doctor diagnoses MS, they will need to identify what type it is and whether it is active or not. The person may need more tests in the future to assess for further changes.

There is no cure for MS, but treatment is available that can:

• slow the progression and reduce the number and severity of relapses
• relieve symptoms

Some people also use complementary and alternative therapies, but research does not always confirm the usefulness of these.

Medications to slow progression

Several disease-modifying therapies (DMTs) have approval from the Food and Drug Administration (FDA) for the relapsing forms of MS. These work by changing the way the immune system functions.

A doctor may give some of these by mouth, some by injection, and some as an infusion. How often the person needs to take them and whether or not they can do this at home will depend on the drug.

The following DMTs currently have approval:

Injectable medications

• interferon beta 1-a (Avonex and Rebif)
• interferon beta-1b (Betaseron and Extavia)
• glatiramer acetate: (Copaxone and Glatopa)
• peginterferon beta-1a) (Plegridy)

Oral medications

• teriflunomide (Aubagio)
• fingolimod (Gilenya)
• dimethyl fumarate (Tecfidera)
• mavenclad (cladribine)
• mayzent (siponimod)

Infused medications

• alemtuzumab (Lemtrada)
• mitoxantrone (Novantrone)
• ocrelizumab (Ocrevus)
• natalizumab (Tysabri)

Current guidelines recommend using these drugs from the early stages, as there is a good chance that they can slow the progression of MS, especially if the person takes them when symptoms are not yet severe.

Some drugs are more useful at specific stages. For example, a doctor may prescribe mitoxantrone at a later, more severe stage of MS.

A doctor will monitor how well a drug is working, as there may be adverse effects, and the same drugs do not suit everyone. New drug options coming onto the market are proving to be safer and more effective than some existing ones.

Adverse effects of immunosuppressant drugs include a higher risk of infections. Some medications may also harm the liver.

If a person notices adverse effects or if their symptoms get worse, they should seek medical advice.

Medications for relieving symptoms during a flare

Other drugs are useful when a person experiences a worsening of symptoms, during a flare. They will not need these drugs all the time.

Corticosteroids: These reduce inflammation and suppress the immune system. They can treat an acute flare-up of symptoms in certain types of MS. Examples include Solu-Medrol (methylprednisolone) and Deltasone (prednisone). Steroids can have adverse effects if a person uses them too often, and they are not likely to provide any long-term benefit.

Behavioral changes: If vision problems occur, a doctor may recommend resting the eyes from time to time or limiting screen time. A person with MS may need to learn to rest when fatigue sets in and to pace themselves so they can complete activities.

Problems with mobility and balance: Physical therapy and walking devices, such as a cane, may help. The drug dalfampridine (Ampyra) may also prove useful.

Tremor: A person may use assistive devices or attach weights to the limbs to reduce shaking. Medications may also help with tremors.

Fatigue: Getting enough rest and avoiding heat can help. Physical and occupational therapy can help teach people more comfortable ways to do things. Assistive devices, such as a mobility scooter, can help conserve energy. Medication or counseling may help boost energy by improving sleep.

Pain: A doctor may prescribe anticonvulsant or antispasmodic drugs or alcohol injections to relieve trigeminal neuralgia, a sharp pain that affects the face. Pain relief medication, such as gabapentin, may help with body pain. There are also medications to relieve muscle pain and cramping in MS.

Bladder and bowel problems: Some medications and dietary changes can help resolve these.

Depression: A doctor may prescribe a selective serotonin reuptake inhibitor (SSRI), as these are less likely to cause fatigue than other antidepressant drugs.

Cognitive changes: Donepezil, a drug for Alzheimer’s, may help some people.

Complementary and alternative therapies

The following may help with different aspects of MS:

• heat and massage treatment for pain
• acupuncture for pain and gait
• stress management to boost mood
• exercise to maintain strength and flexibility, reduce stiffness, and boost mood
• a healthful diet with plenty of fresh fruits, vegetables, and fiber
• quitting or avoiding smoking

Medical marijuana

Studies have suggested that cannabis may help relieve pain, muscle stiffness, and insomnia. However, there is not enough evidence to confirm this.

People should also note that:

• There is a difference between using street cannabis and medical cannabis.
• Not all forms of cannabis are legal in all states.

A person should ask their doctor for advice before using cannabis, as some forms can have adverse effects. Smoking cannabis is unlikely to be beneficial, and it may make symptoms worse.

Rehabilitation and physical therapy

Physical therapy can help with strength and flexibility.

Rehabilitation can help improve or maintain a person’s ability to perform effectively at home and work.

Programs generally include:

Physical therapy: This aims to provide the skills to maintain and restore maximum movement and functional ability.

Occupational therapy: The therapeutic use of work, self-care, and play may help maintain mental and physical function.

Speech and swallowing therapy: A speech and language therapist will carry out specialized training for those who need it.

Cognitive rehabilitation: This helps people manage specific problems in thinking and perception.

Vocational rehabilitation: This helps a person whose life has changed with MS to make career plans, learn job skills, get and keep a job.

Plasma exchange

Plasma exchange involves withdrawing blood from the individual, removing the plasma, replacing it with new plasma, and transfusing it back into the person.

This process removes the antibodies in the blood that are attacking parts of the person’s body, but whether it can help people with MS is unclear. Studies have produced mixed results.

Plasma exchange is usually only suitable for severe MS attacks.

Stem cell therapy

Scientists are looking into the use of stem cell therapy to regenerate various body cells and restore function to those who have lost it due to a health condition.

Researchers hope that one day, stem cell therapy techniques may be able to reverse the damage done by MS and restore functionality in the nervous system.

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INTRODUCTION –

Ankylosing spondylitis is an inflammatory disease that, over time, can cause some of the small bones in your spine (vertebrae) to fuse. This fusing makes the spine less flexible and can result in a hunched-forward posture. If ribs are affected, it can be difficult to breathe deeply.

Ankylosing spondylitis affects men more often than women. Signs and symptoms typically begin in early adulthood. Inflammation also can occur in other parts of your body — most commonly, your eyes.

There is no cure for ankylosing spondylitis, but treatments can lessen your symptoms and possibly slow progression of the disease.

Ankylosing spondylitis (AS) is a rare type of arthritis that causes pain and stiffness in your spine. This lifelong condition, also known as Bechterew disease, usually starts in your lower back. It can spread up to your neck or damage joints in other parts of your body.

“Ankylosis” means fused bones or other hard tissue. “Spondylitis” means inflammation in your spinal bones, or vertebrae. Severe cases can leave your spine hunched.

There’s no cure for AS. But medication and exercise can ease pain and help keep your back strong.

Ankylosing spondylitis (pronounced ank-kih-low-sing spon-dill-eye-tiss),  or AS, is a form of arthritis that primarily affects the spine, although other joints can become involved. It causes inflammation of the spinal joints (vertebrae) that can lead to severe, chronic pain and discomfort.

In more advanced cases this inflammation can lead to ankylosis — new bone formation in the spine — causing sections of the spine to fuse in a fixed, immobile position.

AS can also cause inflammation, pain, and stiffness in other areas of the body such as the shoulders, hips, ribs, heels, and small joints of the hands and feet. Sometimes the eyes can become involved (known as iritis or uveitis), and — rarely — the lungs and heart can be affected.

The hallmark feature of ankylosing spondylitis is the involvement of the sacroiliac (SI) joints during the progression of the disease. The SI joints are located at the base of the spine, where the spine joins the pelvis.

CAUSES-

Ankylosing spondylitis has no known specific cause, though genetic factors seem to be involved. In particular, people who have a gene called HLA-B27 are at a greatly increased risk of developing ankylosing spondylitis. However, only some people with the gene develop the condition.

Although the exact cause of AS is unknown, we do know that genetics play a key role in the disease. Most individuals who have AS also have a gene that produces a “genetic marker,” a protein called HLA-B27. This marker is found in more than 95 percent of people in the Caucasian population with AS. It is important to note, however, that one does not have to be HLA-B27 positive to have AS. Also, a majority of people with this marker never develop ankylosing spondylitis.

Scientists suspect that other genes — along with a triggering environmental factor such as a bacterial infection, for example — are needed to activate AS in susceptible people. HLA-B27 likely accounts for about 30 percent of the overall risk, but there are numerous other genes working in concert with HLA-B27. Researchers have identified more than 60 genes that are associated with AS and related diseases. Among the newer key genes identified are ERAP 1, IL-12, IL-17, and IL-23.

One classic hypothesis has been that AS may start when the defenses of the intestines break down and certain bacteria pass into the bloodstream, triggering changes in the immune response.

The association between ankylosing spondylitis and HLA-B27 varies greatly between ethnic and racial groups.

SYMPTOM-

Early signs and symptoms of ankylosing spondylitis might include pain and stiffness in your lower back and hips, especially in the morning and after periods of inactivity. Neck pain and fatigue also are common. Over time, symptoms might worsen, improve or stop at irregular intervals.

The areas most commonly affected are:

• The joint between the base of your spine and your pelvis
• The vertebrae in your lower back
• The places where your tendons and ligaments attach to bones, mainly in your spine, but sometimes along the back of your heel
• The cartilage between your breastbone and ribs
• Your hip and shoulder joints

When to see a doctor

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Seek medical attention if you have low back or buttock pain that came on slowly, is worse in the morning or awakens you from your sleep in the second half of the night — particularly if this pain improves with exercise and worsens with rest. See an eye specialist immediately if you develop a painful red eye, severe light sensitivity or blurred vision.

RISK FACTOR –

Certain things that you can’t control might raise your risk of AS:

• Sex. Men are more likely than women to have AS, and it strikes them earlier and harder. Women tend to have a milder form of AS called non-radiographic axial spondyloarthritis.
• Age. AS often starts in your teens and young adulthood. About 80% of cases begin before the person turns 30, and 95% by age 45.

• Testing positive for the HLA-B27 marker
• A family history of AS
• Frequent gastrointestinal infections
• Unlike other forms of arthritis and rheumatic diseases, general onset of AS commonly occurs in younger people, between the ages of 17 and 45. However, it can also affect children and those who are much older.

The Centers for Disease Control and Prevention’s NHANES study now estimates that at least 2.7 million adults in the United States have axial spondyloarthritis.

COMPLICATION-

In severe ankylosing spondylitis, new bone forms as part of the body’s attempt to heal. This new bone gradually bridges the gap between vertebrae and eventually fuses sections of vertebrae. Those parts of your spine become stiff and inflexible. Fusion can also stiffen your rib cage, restricting your lung capacity and function.

Other complications might include:

• Eye inflammation (uveitis). One of the most common complications of ankylosing spondylitis, uveitis can cause rapid-onset eye pain, sensitivity to light and blurred vision. See your doctor right away if you develop these symptoms.
• Compression fractures. Some people’s bones thin during the early stages of ankylosing spondylitis. Weakened vertebrae can crumble, increasing the severity of your stooped posture. Vertebral fractures can put pressure on and possibly injure the spinal cord and the nerves that pass through the spine.
• Heart problems. Ankylosing spondylitis can cause problems with your aorta, the largest artery in your body. The inflamed aorta can enlarge to the point that it distorts the shape of the aortic valve in the heart, which impairs its function.

DIAGNOSIS-

A rheumatologist is commonly the type of physician who will diagnose ankylosing spondylitis (AS), since they are doctors who are specially trained in diagnosing and treating disorders that affect the joints, muscles, tendons, ligaments, connective tissue, and bones. A thorough physical exam, including X-rays, individual medical history, and a family history of AS, as well as blood work (including a test for HLA-B27) are factors in making a diagnosis.

AS can be tough to spot because so many people have back pain, its main symptom. A diagnosis may be even trickier for women because the condition is much more common in men.

There’s also no single test to confirm AS. Your doctor may rely on your symptoms, a physical exam, and blood tests.

You also might have an X-ray or an MRI. But this doesn’t always help, because joint damage may not show up right away on imaging tests.

TREATMENT-

Medications help some people. But staying active is one of the keys to managing AS.

Exercise. The less you sit or lie down, the better you’ll feel. Exercise helps you stand straighter and keeps your spine limber. Staying active may even banish your pain without medication.

Physical therapy. You’ll need to practice good posture, learn how to stretch tight muscles and keep your spine stable, and use other techniques that can lower your pain. You can do them at home, but most people benefit more from working with a professional physical therapist or with a group.

Medication

Prescription nonsteroidal anti-inflammatory drugs (NSAIDs) like indomethacin (Indocin) help most people who have AS. But they can lead to stomach bleeding, heart problems, and other side effects.

If your condition is severe, your doctor may have you try stronger medications like biologics. These are made with things like proteins. But they may cause serious side effects, including infection.

Ankylosing Spondylitis Lifestyle and Home Remedies

Some things you do every day can help you feel better.

• Make time to exercise every day, even a few minutes at a time. Working out in water helps a lot of people who have AS.
• Keep a healthy weight so your joints aren’t under as much stress. A diet high in omega-3 fatty acids might help. Watch for patterns if you think certain foods might trigger changes in how you feel.
• Don’t smoke. People who smoke tobacco often have symptoms that get worse as they get older.
• Manage stress with things like massage, yoga, meditation, and counseling.
• Apply heat to stiff joints and tight muscles, and use cold on inflamed areas.

PROGNOSIS-

The severity of AS varies greatly from person to person, and not everyone will experience the most serious complications or have spinal fusion. Some may experience only intermittent back pain and discomfort, while others may experience severe pain and stiffness over multiple areas of the body for long periods of time. AS can be debilitating and, in some cases, lead to disability.

Almost all cases of AS are characterized by acute, painful episodes (also known as “flares”), which are followed by temporary periods of remission when symptoms subside.

It is important to know that ankylosing spondylitis is a chronic, or lifelong, disease and that the severity of AS has nothing to do with age or gender. It can be just as severe in women and children as in men.

Remember that even if you have AS and are experiencing only mild symptoms, which you are able to manage well, it is important to see your rheumatologist once a year in order to detect and treat any underlying complications.

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Epidemiology

• There is a strong female predilection in adults, with women affected 9-13 times more than males. In children, this ratio is reversed, and males are affected two to three times more often.
• Can affect any age group – the peak age at onset around the 2nd to 4th decades, with 65% of patients presenting between the ages of 16 and 65 years (i.e. during childbearing years).
• Disease is more common in childbearing age in women however it has been well reported in the pediatric and elderly population. SLE is more severe in children while in the elderly, it tends to be more insidious onset and has more pulmonary involvement and serositis and less Raynaud’s, malar rash, nephritis, and neuropsychiatric complications
• Studies have indicated that although rare, lupus in men tends to be more severe.
• Prevalence varies according to ethnicity with ratios as high as 1:500 to 1:1000 in Afro-Caribbeans and indigenous Australians, down to 1:2000 in Caucasians.

How can systemic lupus erythematosus affect pregnancy or the newborn?

Lupus pregnancy deserves special review because it presents unique challenges. Pregnant women with SLE are considered high-risk pregnancies. These pregnancies require interactive monitoring generally by a skilled rheumatologist together with an obstetrician expert in high-risk pregnancies. Women with SLE who are pregnant require close observation during pregnancy, delivery, and the postpartum period. This includes fetal monitoring by the obstetrician during later pregnancy. These women can have an increased risk of miscarriages (spontaneous abortions) and can have flares of SLE during pregnancy. The presence of phospholipid antibodies, such as cardiolipin antibodies or lupus anticoagulant, in the blood can identify people at risk for miscarriages. Cardiolipin antibodies are associated with a tendency toward blood clotting. Women with SLE who have cardiolipin antibodies or lupus anticoagulant may need blood-thinning medications (aspirin with or without heparin) during pregnancy to prevent miscarriages. Other reported treatments include the use of intravenous gamma globulin for selected people with histories of premature miscarriage and those with low blood-clotting elements (platelets) during pregnancy.

Lupus antibodies can be transferred from the mother to the fetus and result in lupus illness in the newborn (“neonatal lupus”). This includes the development of low red cell counts (hemolytic anemia) and/or white blood cell counts (leucopenia) and platelet counts (thrombocytopenia) and skin rash. Problems can also develop in the electrical system of the baby’s heart (congenital heart block). Occasionally, a pacemaker for the baby’s heart is needed in this setting. Neonatal lupus and congenital heart block are more common in newborns of mothers with SLE who carry specific antibodies referred to as anti-Ro (or anti-SSA) and anti-La (or anti-SSB). (It is helpful for the newborn baby’s doctor to be made aware if the mother is known to carry these antibodies, even prior to delivery. The risk of heart block is 2%; the risk of neonatal lupus is 5%.) Neonatal lupus usually clears after 6 months of age, as the mother’s antibodies are slowly metabolized by the baby.

PROGNOSIS-

Overall, the outlook for people living with systemic lupus is improving each decade with the development of more accurate monitoring tests and treatments.

The role of the immune system in causing diseases is becoming better understood through research. This knowledge will be applied to design safer and more effective treatment methods. For example, completely revising the immune system of people with extremely aggressive treatments that virtually temporarily wipe out the immune system is being evaluated. Current studies involve immune eradication with or without replacement of cells that can reestablish the immune system (stem-cell transplantation).

It should be noted that people with SLE are at a somewhat increased risk for developing cancer. The cancer risk is most dramatic for blood cancers, such as leukemia and lymphoma, but is also increased for breast cancer. This risk probably relates, in part, to the altered immune system that is characteristic of SLE.

Women with SLE appear to be at increased risk for heart disease (coronary artery disease) according to recent reports. Women with SLE should be evaluated and counseled to minimize risk factors for heart disease, such as elevated blood cholesterol, quitting smoking, high blood pressure, and obesity.

Landmark research has shown clearly that oral contraceptives do not increase the rate of flares of systemic lupus erythematosus. This important finding is opposite to what has been thought for years. Now we can reassure women with lupus that if they take birth-control pills, they are not increasing their risk for lupus flares. Note: Women who are at increased risk of blood clotting, such as women with lupus who have phospholipid antibodies (including cardiolipin antibody and lupus anticoagulant), should avoid birth-control pills or any estrogen medications.

Individuals living with SLE can improve their long-term prognosis by learning about the many aspects of the illness as well as closely monitoring their own health with their doctors.

TREATMENT-

No cure for SLE exists. The goal of treatment is to ease symptoms. Treatment can vary depending on how severe your symptoms are and which parts of your body SLE affects. The treatments may include:

• anti-inflammatory medications for joint pain and stiffness, such as these options available online
• steroid creams for rashes
• corticosteroids to minimize the immune response
• antimalarial drugs for skin and joint problems
• disease modifying drugs or targeted immune system agents for more severe cases

Talk with your doctor about your diet and lifestyle habits. Your doctor might recommend eating or avoiding certain foods and minimizing stress to reduce the likelihood of triggering symptoms. You might need to have screenings for osteoporosis since steroids can thin your bones. Your doctor may also recommend preventive care, such as immunizations that are safe for people with autoimmune diseases and cardiac screenings,

PHYSICAL THERAPY FOR S.L.E.

Exercise is beneficial for patients with SLE because it decreases their muscle weakness while simultaneously increases their muscle endurance. Physical therapists can play an important role for patients with SLE during and between exacerbations.  The patient’s need for physical therapy will vary greatly depending on the systems involved.   

• Education: It is essential for patients with skin lesions to have appropriate education on the best way to care for their skin and to ensure they do not experience additional skin breakdown.  
• Aerobic Exercise:  One of the most common impairments that patients with SLE experience is generalized fatigue that can limit their activities throughout the day.  Graded aerobic exercise programs are more successful than relaxation techniques in decreasing the fatigue levels of patients with SLE.  Aerobic activity causes many with SLE to feel much better.  The aerobic exercise program may consisted of 30-50 minutes of aerobic activity (walking/swimming/cycling) with a heart rate corresponding to 60% of the patient’s peak oxygen consumption.  Both aerobic exercise and range of motion/muscle strengthening exercises can increase the energy level, cardiovascular fitness, functional status, and muscle strength in patients with SLE (aerobic exercise for 20-30 minutes at 70-80% of their maximum heart rate,3 times a week for 50 minutes sessions).
• Energy Conservation: Physical therapists can educate patients on appropriate energy conservation techniques and the best ways to protect joints that are susceptible to damage.  
• Additionally, physical therapists and patients with SLE should be aware of signs and symptoms that suggest a progression of SLE including those associated with avascular necrosis, kidney involvement, and neurological involvement.

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INTRODUCTION-

Systemic lupus erythematosus (SLE), is the most common type of lupus. SLE is an autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs. It can affect the joints, skin, brain, lungs, kidneys, and blood vessels. There is no cure for lupus, but medical interventions and lifestyle changes can help control it.

The immune system normally fights off dangerous infections and bacteria to keep the body healthy. An autoimmune disease occurs when the immune system attacks the body because it confuses it for something foreign. There are many autoimmune diseases, including systemic lupus erythematosus (SLE).

The term lupus has been used to identify a number of immune diseases that have similar clinical presentations and laboratory features, but SLE is the most common type of lupus. People are often referring to SLE when they say lupus.

SLE is a chronic disease that can have phases of worsening symptoms that alternate with periods of mild symptoms. Most people with SLE are able to live a normal life with treatment.

According to the Lupus Foundation of America, at least 1.5 million Americans are living with diagnosed lupus. The foundation believes that the number of people who actually have the condition is much higher and that many cases go undiagnosed.Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by antibodies to nuclear and cytoplasmic antigens, multisystem inflammation, protean clinical manifestations, and a relapsing and remitting course. More than 90% of cases of SLE occur in women, frequently starting at childbearing age. See the image below.

CAUSES-

The exact cause of SLE isn’t known, but several factors have been associated with the disease.

Genetics

The disease isn’t linked to a certain gene, but people with lupus often have family members with other autoimmune conditions.

Environment

Environmental triggers can include:

• ultraviolet rays
• certain medications
• viruses
• physical or emotional stress
• trauma

Sex and hormones

SLE affects women more than men. Women also may experience more severe symptoms during pregnancy and with their menstrual periods. Both of these observations have led some medical professionals to believe that the female hormone estrogen may play a role in causing SLE. However, more research is still needed to prove this theory.

DIAGNOSIS-

Your doctor will do a physical exam to check for typical signs and symptoms of lupus, including:

• sun sensitivity rashes, such as a malar or butterfly rash
• mucous membrane ulcers, which may occur in the mouth or nose
• arthritis, which is swelling or tenderness of the small joints of the hands, feet, knees, and wrists
• hair loss
• hair thinning
• signs of cardiac or lung involvement, such as murmurs, rubs, or irregular heartbeats

No one single test is diagnostic for SLE, but screenings that can help your doctor come to an informed diagnosis include:

• blood tests, such as antibody tests and a complete blood count
• a urinalysis
• a chest X-ray

Your doctor might refer you to a rheumatologist, which is a doctor who specializes in treating joint and soft tissue disorders and autoimmune diseases.

Lupus is a chronic disease with no cure. This means that you can manage it with treatment, but it will not go away. Treatment can help improve your symptoms, prevent flares, and prevent other health problems often caused by lupus. Your treatment will depend on your symptoms and needs.

Lupus can be hard to diagnose because it has many symptoms that are often mistaken for symptoms of other diseases. Many people have lupus for a while before they find out they have it. If you have symptoms of lupus, tell your doctor right away.

No single test can tell if a person has lupus. But your doctor can find out if you have lupus in other ways, including:

• Medical history. Tell your doctor about your symptoms and other problems. Keep track of your symptoms by writing them down when they happen. Also, track how long they last.
• Family history of lupus or other autoimmune diseases. Tell your doctor if lupus or other autoimmune diseases run in your family.
• Complete physical exam. Your doctor will look for rashes and other signs that something is wrong.
• Blood and urine tests. The antinuclear antibody (ANA) test can show if your immune system is more likely to make the autoantibodies of lupus. Most people with lupus test positive for ANA. But, a positive ANA does not always mean you have lupus. If you test positive for ANA, your doctor will likely order more tests for antibodies that are specific to systemic lupus erythematosus (SLE).
• Skin or kidney biopsy. A biopsy is a minor surgery to remove a sample of tissue. The tissue is then viewed under a microscope. Skin and kidney tissue looked at in this way can show signs of an autoimmune disease.

Your doctor may use any or all of these tests to make your diagnosis. They also can help your doctor rule out other diseases that can be confused with lupus.

SIGNS AND SYMPTOM-

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Symptoms of systemic lupus erythematosus (SLE, often referred to as simply lupus) can be quite different, because there are different types of lupus, and not all affected people show the same symptoms.

General symptoms associated with lupus include

• low-grade fever,
• loss of appetite,
• nausea,
• muscle aches,
• joint pains,
• fatigue.

More specific symptoms include skin changes (see below), ulcers of the mouth and nose, photosensitivity (sensitivity to sunlight), and decreased circulation to the fingers and toes with cold exposure.

Complications of organ involvement can lead to further symptoms that depend on the organ affected and severity of the disease.

SLE-associated skin manifestations can sometimes lead to scarring. In discoid lupus, only the skin is typically involved. The skin rash in discoid lupus often is found on the face and scalp. It usually is red and may have raised borders. Discoid lupus rashes are usually painless and do not itch, but scarring can cause permanent hair loss (alopecia). Over time, 5%-10% of those with discoid lupus may develop SLE.

Over half of the people with SLE develop a characteristic red, flat facial rash over the bridge of their nose. Because of its shape, it is frequently referred to as the “butterfly rash” of SLE. The rash is painless and does not itch. The facial rash, along with inflammation in other organs, can be precipitated or worsened by exposure to sunlight, a condition called photosensitivity. This photosensitivity can be accompanied by worsening of inflammation throughout the body, called a “flare” of the disease.

Typically, with treatment, this rash can heal without permanent scarring.

Most SLE patients will develop arthritis during the course of their illness. Arthritis from SLE commonly involves swelling, pain, stiffness, and even deformity of the small joints of the hands, wrists, and feet. Sometimes, the arthritis of SLE can mimic that of rheumatoid arthritis (another autoimmune disease).

More serious organ involvement with inflammation occurs in the brain, liver, and kidneys. White blood cells can be decreased in SLE (referred to as leukopenia or leucopenia). Also, low blood-clotting factors called platelets (thrombocytopenia) can be caused by lupus. Leukopenia can increase the risk of infection, and thrombocytopenia can increase the risk of bleeding. Low red blood cell counts (hemolytic anemia) can occur.

Inflammation of muscles (myositis) can cause muscle pain and weakness. This can lead to elevations of muscle enzyme levels in the blood.

Inflammation of blood vessels (vasculitis) that supply oxygen to tissues can cause isolated injury to a nerve, the skin, or an internal organ. The blood vessels are composed of arteries that pass oxygen-rich blood to the tissues of the body and veins that return oxygen-depleted blood from the tissues to the lungs. Vasculitis is characterized by inflammation with damage to the walls of various blood vessels.

he damage blocks the circulation of blood through the vessels and can cause injury to the tissues that are supplied with oxygen by these vessels.

Inflammation of the lining of the lungs (pleuritis) with pain aggravated by deep breathing (pleurisy) and of the heart (pericarditis) can cause sharp chest pain. The chest pain is aggravated by coughing, deep breathing, and certain changes in body position. The heart muscle itself rarely can become inflamed (carditis). It has also been shown that young women with SLE have a significantly increased risk of heart attacks due to coronary artery disease.

Kidney inflammation in SLE (lupus nephritis) can cause leakage of protein into the urine, fluid retention, high blood pressure, and even kidney failure. This can lead to further fatigue and swelling (edema) of the legs and feet. With kidney failure, machines are needed to cleanse the blood of accumulated waste products in a process called dialysis.

Involvement of the brain can cause personality changes, thought disorders (psychosis), seizures, and even coma. Lupus of the nervous system (neurologic lupus) can lead to damage to nerves cause numbness, tingling, and weakness of the involved body parts or extremities. Brain involvement is referred to as lupus cerebritis.

Many people with SLE experience hair loss (alopecia). Often, this occurs simultaneously with an increase in the activity of their disease. The hair loss can be patchy or diffuse and appear to be more like hair thinning.

Some people with SLE have Raynaud’s phenomenon. Raynaud’s phenomenon causes the blood vessels of the hands and feet to spasm, especially upon exposure to cold. The blood supply to the fingers and/or toes then becomes compromised, causing blanching, whitish and/or bluish discoloration, and pain and numbness in the exposed fingers and toes.

Other diseases and conditions that can accompany lupus include fibromyalgia, coronary heart disease, nonbacterial valvular heart disease, pancreatitis, esophagus disease with difficulty swallowing (dysphagia), swollen lymph nodes (lymphadenopathy), liver disease (lupoid hepatitis), infections, and a tendency to spontaneous blood clotting and thrombosis.

Recognizing potential symptoms of SLE

Symptoms can vary and can change over time. Common symptoms include:

• severe fatigue
• joint pain
• joint swelling
• headaches
• a rash on the cheeks and nose, which is called a “butterfly rash”
• hair loss
• anemia
• blood-clotting problems
• fingers turning white or blue and tingling when cold, which is known as Raynaud’s phenomenon

Other symptoms depend on the part of the body the disease is attacking, such as the digestive tract, the heart, or the skin.

Lupus symptoms are also symptoms of many other diseases, which makes diagnosis tricky. If you have any of these symptoms, see your doctor. Your doctor can run tests to gather the information needed to make an accurate diagnosis.

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RISK FACTORS-

The precise reason for the abnormal autoimmunity that causes lupus is not known. Inherited genes, viruses, ultraviolet light, and certain medications may all play some role.

Lupus is not caused by an infectious microorganism and is not contagious from one person to another.

Genetic factors increase the tendency of developing autoimmune diseases, and autoimmune diseases such as lupus, rheumatoid arthritis, and autoimmune thyroid disorders are more common among relatives of people with lupus than the general population. Moreover, it is possible to have more than one autoimmune disease in the same individual. Therefore, “overlap” syndromes of lupus and rheumatoid arthritis, or lupus and scleroderma, etc., can occur.

Some scientists believe that the immune system in lupus is more easily stimulated by external factors like viruses or ultraviolet light. Sometimes, symptoms of lupus can be precipitated or aggravated by only a brief period of sun exposure.

It also is known that some women with systemic lupus erythematosus can experience worsening of their symptoms prior to their menstrual periods. This phenomenon, together with the female predominance of systemic lupus erythematosus, suggests that female hormones play an important role in the expression of SLE. This hormonal relationship is an active area of ongoing study by scientists.

Research has demonstrated evidence that a key enzyme’s failure to dispose of dying cells may contribute the development of systemic lupus erythematosus. The enzyme, DNase1, normally eliminates what is called “garbage DNA” and other cellular debris by chopping them into tiny fragments for easier disposal. Researchers turned off the DNase1 gene in mice. The mice appeared healthy at birth, but after six to eight months, the majority of mice without DNase1 showed signs of systemic lupus erythematosus. Thus, a genetic mutation in a gene that could disrupt the body’s cellular waste disposal may be involved in the initiation of systemic lupus erythematosus.

COMPLICATION-

Over time, SLE can damage or cause complications in systems throughout your body. Possible complications may include:

• blood clots and inflammation of blood vessels or vasculitis
• inflammation of the heart, or pericarditis
• a heart attack
• a stroke
• memory changes
• behavioral changes
• seizures
• inflammation of lung tissue and the lining of the lung, or pleuritis
• kidney inflammation
• decreased kidney function
• kidney failure

SLE can have serious negative effects on your body during pregnancy. It can lead to pregnancy complications and even miscarriage. Talk with your doctor about ways to reduce the risk of complications.

DRUG INDUCED LUPUS-

Dozens of medications have been reported to trigger SLE. However, more than 90% of cases of “drug-induced lupus” occurs as a side effect of one of the following six drugs: hydralazine (Apresoline) is used for high blood pressure; quinidine (Quinidine Gluconate, Quinidine Sulfate) and procainamide (Pronestyl; Procan-SR; Procanbid) are used for abnormal heart rhythms; phenytoin (Dilantin) is used for epilepsy; isoniazid (Nydrazid, Laniazid) is used for tuberculosis; and d-penicillamine (used for rheumatoid arthritis.

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INTRODUCTION-

Rickets is a childhood bone condition wherein the bones soften and become prone to fractures and irregularities. The main cause of rickets is a lack of vitamin D, but people can also inherit a certain type of rickets.

Rickets is a skeletal disorder that’s caused by a lack of vitamin D, calcium, or phosphate. These nutrients are important for the development of strong, healthy bones. People with rickets may have weak and soft bones, stunted growth, and, in severe cases, skeletal deformities.

Vitamin D helps your body absorb calcium and phosphate from your intestines. You can get vitamin D from various food products, including milk, eggs, and fish. Your body also produces the vitamin when you’re exposed to sunlight.

A vitamin D deficiency makes it difficult for your body to maintain sufficient levels of calcium and phosphate. When this occurs, your body produces hormones that cause calcium and phosphate to be released from your bones. When your bones lack these minerals, they become weak and soft.

Rickets is most common in children who are between 6 and 36 months old. Children are at the highest risk of rickets because they’re still growing. Children might not get enough vitamin D if they live in a region with little sunlight, follow a vegetarian diet, or don’t drink milk products. In some cases, the condition is hereditary.

Rickets is rare in the United States. Rickets used to be more common, but it mostly disappeared in developed countries during the 1940s due to the introduction of fortified foods, such as cereals with added vitamin D.

Rickets is the softening and weakening of bones in children, usually because of an extreme and prolonged vitamin D deficiency. Rare inherited problems also can cause rickets.

Vitamin D helps your child’s body absorb calcium and phosphorus from food. Not enough vitamin D makes it difficult to maintain proper calcium and phosphorus levels in bones, which can cause rickets.

Adding vitamin D or calcium to the diet generally corrects the bone problems associated with rickets. When rickets is due to another underlying medical problem, your child may need additional medications or other treatment. Some skeletal deformities caused by rickets may require corrective surgery.

Rare inherited disorders related to low levels of phosphorus, the other mineral component in bone, may require other medications.

Rickets is rare in populations whose governments require certain foods to have added vitamin D. However, there are concerns that the number of cases has risen in the United States since 2000.

Vitamin D plays a vital role in calcium absorption, so very low vitamin D levels can lead to low calcium levels.

As a result, developing bones can become weak and may form irregularly. People may also experience bone pain. The resulting symptoms can persist into adulthood. A severe vitamin D deficiency in adulthood can lead to osteomalacia, which is similar to rickets.

A vitamin D deficiency may result from a low dietary intake of vitamin D or low exposure to or absorption of ultraviolet (UV) rays. This means that children who spend a lot of time indoors may be at risk of vitamin D deficiency and rickets.

Rickets can also result from some metabolic and genetic conditions.

Taking vitamin D supplements may help protect those at risk.

CAUSES-

There are several causes of rickets, including:

Lack of vitamin D

The human body needs vitamin D to absorb calcium from the intestines. UV rays from sunlight help the skin cells convert a precursor of vitamin D from an inactive to an active state.

Children who don’t get enough vitamin D from these two sources can develop a deficiency:

• Sunlight. Your child’s skin produces vitamin D when it’s exposed to sunlight. But children in developed countries tend to spend less time outdoors. They’re also more likely to use sunscreen, which blocks the sun’s rays that trigger the skin’s production of vitamin D.
• Food. Fish oil, egg yolks and fatty fish such as salmon and mackerel contain vitamin D. Vitamin D has also been added to some foods and beverages, such as milk, cereal and some fruit juices.

If a person does not make or consume enough vitamin D, their body may not absorb sufficient calcium from the food they eat, causing low levels of calcium in the blood.

Low calcium levels result in irregularities of the bones and teeth, as well as nerve and muscle problems.

Children may lack vitamin D if they:

• have dark skin
• spend a lot of time indoors
• always wear sunscreen when outside
• follow a lactose-free or strict plant-based diet
• have a health condition such as celiac disease, which prevents the body from making or using vitamin D
• live in a place with high levels of air pollution

Regarding infants, the Centers for Disease Control and Prevention (CDC) note that breast milk does not provide enough vitamin D. According to the CDC, the American Academy of Pediatrics recommend vitamin D supplements of 400 international units (IU) (10 micrograms [mcg]) for infants who are wholly or partially breastfed. Formula milk tends to be fortified with vitamin D.

Genetic factors

Some types of rickets result from a genetic condition. These may be hereditary.

Hypophosphatemic rickets, for example, is a rare condition in which the kidneys are unable to process phosphate properly. Low levels of phosphate in the blood lead to weak and soft bones.

The most common type affects around 1 in 20,000 newborns.

Genetic factors that affect the body’s ability to use calcium can result in rickets, including those that affect liver, kidney, and intestinal function.

Calcium is also important for bone strength. Find out which foods provide calcium here.

Problems with absorption

Some children are born with or develop medical conditions that affect the way their bodies absorb vitamin D. Some examples include:

• Celiac disease
• Inflammatory bowel disease
• Cystic fibrosis
• Kidney problems

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SYMPTOM-

Signs and symptoms of rickets can include:

• Delayed growth
• Delayed motor skills
• Pain in the spine, pelvis and legs
• Muscle weakness

Because rickets softens the areas of growing tissue at the ends of a child’s bones (growth plates), it can cause skeletal deformities such as:

• Bowed legs or knock knees
• Thickened wrists and ankles
• Breastbone projection

When to see a doctor

Talk to your doctor if your child develops bone pain, muscle weakness or obvious skeletal deformities.

RISK FACTOR-

Factors that can increase a child’s risk of rickets include:

• Dark skin. Dark skin has more of the pigment melanin, which lowers the skin’s ability to produce vitamin D from sunlight.
• Mother’s vitamin D deficiency during pregnancy. A baby born to a mother with severe vitamin D deficiency can be born with signs of rickets or develop them within a few months after birth.
• Northern latitudes. Children who live in geographical locations where there is less sunshine are at higher risk of rickets.
• Premature birth. Babies born before their due dates tend have lower levels of vitamin D because they had less time to receive the vitamin from their mothers in the womb.
• Medications. Certain types of anti-seizure medications and antiretroviral medications, used to treat HIV infections, appear to interfere with the body’s ability to use vitamin D.
• Exclusive breast-feeding. Breast milk doesn’t contain enough vitamin D to prevent rickets. Babies who are exclusively breast-fed should receive vitamin D drops.

Age

Rickets is most common in children who are between 6 and 36 months old. During this time period, children usually experience rapid growth. This is when their bodies need the most calcium and phosphate to strengthen and develop their bones.

Diet

You have a higher risk of developing rickets if you eat a vegetarian diet that doesn’t include fish, eggs, or milk. You’re also at an increased risk if you have trouble digesting milk or have an allergy to milk sugar (lactose). Infants who are only fed breast milk can become deficient in vitamin D as well. Breast milk doesn’t contain enough vitamin D to prevent rickets.

Skin color

Children of African, Pacific Islander, and Middle Eastern descent are at the highest risk for rickets because they have dark skin. Dark skin doesn’t react as strongly to sunlight as lighter skin does, so it produces less vitamin D.

Geographic location

Our bodies produce more vitamin D when they’re exposed to sunshine, so you’re more at risk for rickets if you live in an area with little sunlight. You’re also at a higher risk if you work indoors during daylight hours.

Genes

One form of rickets can be inherited. This means that the disorder is passed down through your genes. This type of rickets, called hereditary rickets, prevents your kidneys from absorbing phosphate.

COMPLICATION-

Left untreated, rickets can lead to:

• Failure to grow
• An abnormally curved spine
• Bone deformities
• Dental defects
• Seizures

DIAGNOSIS-

Your doctor may be able to diagnose rickets by performing a physical examination. They will check for tenderness or pain in the bones by lightly pressing on them. Your doctor may also order certain tests to help make a rickets diagnosis, including:

• blood tests to measure the levels of calcium and phosphate in the blood
• bone X-rays to check for bone deformities

In rare cases, a bone biopsy will be performed. This involves the removal of a very small section of bone, which will be sent to a laboratory for analysis.

PREVENTION-

Exposure to sunlight provides the best source of vitamin D. During most seasons, 10 to 15 minutes of exposure to the sun near midday is enough. However, if you’re dark-skinned, if it’s winter or if you live in northern latitudes, you might not be able to get enough vitamin D from sun exposure.

In addition, because of skin cancer concerns, infants and young children, especially, are warned to avoid direct sun or to always wear sunscreen and protective clothing.

To prevent rickets, make sure your child eats foods that contain vitamin D naturally — fatty fish such as salmon and tuna, fish oil and egg yolks — or that have been fortified with vitamin D, such as:

• Infant formula
• Cereal
• Bread
• Milk, but not foods made from milk, such as some yogurts and cheese
• Orange juice

Check labels to determine the vitamin D content of fortified foods.

If you’re pregnant, ask your doctor about taking vitamin D supplements.

Guidelines recommend that all infants should receive 400 IU a day of vitamin D. Because human milk contains only a small amount of vitamin D, infants who are exclusively breast-fed should receive supplemental vitamin D daily. Some bottle-fed infants may also need vitamin D supplements if they aren’t receiving enough from their formula.

TREATMENT-

Treatment will aim to maximize the individual’s intake of calcium, phosphate, and vitamin D.

Depending on the underlying cause, a doctor will usually prescribe vitamin D supplements.

They may also recommend:

• increasing exposure to sunlight
• making dietary changes
• taking fish oil
• getting more exposure to UVB light
• consuming calcium and phosphorus

Get more information on vitamin D and joint pain here.

Dietary measures

If rickets results from a poor diet, a doctor may prescribe:

• daily calcium and vitamin D supplements
• an annual vitamin D injection (if a person cannot take supplements orally)
• a diet plan that focuses on foods rich in vitamin D

To add vitamin D to the diet, a person can consume:

• eggs
• cod liver oil
• oily fish, such as salmon, tuna, sardines, and swordfish
• vitamin D-fortified foods, such as milk, some juices, many cereals, some brands of margarine, and some soy milk products
• beef liver

Making dietary changes and spending some time outside each day can help prevent rickets in most children.

Treating medical causes

If the cause is genetic, a doctor may prescribe phosphate and calcitriol supplements to reduce bowing in the legs.

If there is an underlying medical cause, such as kidney disease, treating it may help prevent rickets.

PHYSICAL THERAPY FOR RICKETS –

Physical therapists can take a team approach with medical management through patient education on: Foods high in vitamin D; Importance of following medical recommendations for vitamin D intake; Importance of proper sun exposure with risks of overexposure. A study suggests that implementing a fall treatment protocol comprised of a multidisciplinary team of a Family Medicine (FM) physician, an Internal Medicine (IM) physician, a physical therapist, and a Home Health (HH) nurse leads to more consistent care of elderly patients who experience falls. However, there is a need for reviewing and updating the protocol based on outcomes, and subsequent research is required for improvement in the patient care.

There are no direct physical therapy interventions for vitamin D deficiency. Patient will be referred to physical therapy for treatment of impairments that may be a cause of vitamin D deficiency such as decline in muscle strength, decline in physical functioning, or falls prevention. (See Clinical Presentation). In these instances techniques could include:

Falls prevention training eg Otago program, and falls exercise classes

General muscle strengthening exercises

In older adults, there is a blunted responsiveness to resistance training and reduced muscle hypertrophy compared with younger adults. There is evidence that both exercise training and vitamin D supplementation may benefit musculoskeletal health in older adults, and it is plausible that in combination their effects may be additive. Vitamin D deficiency is associated with impaired muscle strength and performance in community-dwelling older people.

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Therapy

Benzodiazepines

Benzodiazepines (BZDs) act by modulating the binding of GABA to the GABA‐A receptor, increasing the influx of chloride ions and providing an inhibitory effect which is similar to that of ethanol. Therefore, BZDs replace the repressive effect of ethanol that has been discontinued in AWS. Most BZDs are extensively and rapidly absorbed after oral administration, with bioavailability varying from 80% to 100%. They rapidly penetrate the blood–brain barrier, although the diffusion rate into the brain and other tissues varies and is largely determined by lipophilicity. All BZDs are metabolized in the liver by oxidation and/or glucuronidation, and some of them form pharmacologically active metabolites that are responsible for the long duration of action, such as diazepam, chlordiazepoxide, and clorazepate. Therefore, the BZDs and their active metabolites may be categorized according to the duration of their effect: short acting (<10 h like lorazepam, oxazepam, and midazolam), intermediate acting (10–24 h as clonazepam), or long acting (>24 h; clobazam, clorazepate, and diazepam).. The metabolism of BZDs is primarily catalyzed by CYP isoenzymes which may be the target of drug–drug interactions, sometimes leading to paradoxical effects or over sedation. When associated with paradoxical excitement, BZDs may contribute to seizure exacerbation when tapered, particularly after prolonged use.

BZDs are currently recognized as first‐line treatment for AWS. Their effectiveness to significantly reduce the risk of recurrent seizures related to alcohol withdrawal compared to placebo has been demonstrated many years ago. Nevertheless, the available evidence does not suggest that benzodiazepines are clearly superior to other drugs with the exception of a possible advantage in seizure control and prevention when compared to non‐anticonvulsants and placebo. BZDs are recommended both for primary and secondary seizure prophylaxis in AWS. A structured guideline for the identification and management of alcohol‐related seizures (EFNS TaskForce, 2005) is currently being revised. Within the first 2 d of withdrawal, BZDs reduce the incidence of seizures by up to 84% and prevent the development of DT. The current literature does not suggest one BZD to be more efficacious than another, although differences in pharmacokinetic properties can guide selection. The following recommendations include agents

1. with rapid onset to control agitation symptoms
2. with long action to avoid breakthrough symptoms
3. with less dependence on hepatic metabolism to lower the risk of over sedation

Diazepam fulfills the first two aspects and represents the primary choice. Increased age and liver disease significantly impact the CYP‐dependent metabolism of medications with a 50% decline in the clearance and a four‐ to ninefold increase in terminal half‐life of diazepam with accumulation and production of side effects. Therefore, in the elderly and patients with cirrhosis or severe liver dysfunction, lorazepam or oxazepam is preferred.

Strategies for the use of BDZ

Multiple dosing strategies have been utilized in the management of AWS. When using any dosing technique, it is important to recognize the symptoms of benzodiazepine toxicity that can include respiratory depression, excessive sedation, ataxia, confusion, memory impairment, and delirium, which may be difficult to differentiate from DT .

Loading dose regimen

The “front‐loading” or “loading dose” strategy uses high doses of longer‐acting benzodiazepines to quickly achieve initial sedation with a self‐tapering effect over time due to their pharmacokinetic properties. Typically, diazepam 10–20 mg or chlordiazepoxide 100 mg doses are repeated every 1–2 h until the patient reaches adequate sedation with an average of three doses usually required. Studies found diazepam loading to significantly reduce the risk of complications, to reduce the total dose of benzodiazepines needed, and the duration of withdrawal symptoms. A further benefit of this approach is that intensive monitoring and medication administration are limited to the early period of withdrawal. As the loading dose regimen may cause sedation and respiratory depression, withdrawal severity and the clinical condition need to be monitored prior to each dose to avoid benzodiazepine toxicity. This is especially important in elderly patients and those with hepatic dysfunction.

Fixed‐dose application

The “fixed‐dose” technique implies that a certain amount of medication is administered at regular intervals. This approach may be beneficial for patients who will require medication regardless of symptoms, such as in those with a history of seizures or DT.3 Fixed‐schedule dosing is often the only way to treat patients withdrawing from alcohol with comorbid medical illnesses or SE because of inability to assess withdrawal symptoms. Other advantages are less frequent reassessments of symptoms and fewer protocol errors in comparison with the symptom‐triggered therapy.Chlordiazepoxide and diazepam remain the agents of choice because of their long‐acting nature. A ceiling dose of 60 mg of diazepam or 125 mg of chlordiazepoxide is advised per day. After 2–3 d of stabilization of the withdrawal syndrome, the benzodiazepine is gradually tapered off over a period of 7–10 d.The peril of the fixed‐dose regimen is seen in under‐ or overestimation of the total dose; the latter is often seen in patients who are still alcohol intoxicated where unpredictable interactions with BZD may emerge.

Symptom‐triggered treatment

For this approach to be successful, patients must be symptomatic and there must be regular assessment of patient’s withdrawal symptoms using a validated tool like the CIWA‐Ar scale. Therefore, this regimen requires close monitoring. For this reason, the technique is not applicable in non‐verbal patients, and it is not safe in patients with a past history of withdrawal seizures because they can occur even without AWS symptoms. Using CIWA‐Ar, the cutoff for beginning treatment is a score of at least 8 resulting in the application of 5–10 mg diazepam or 25–100 mg chlordiazepoxide. Assessment should be repeated 1 h later. If symptoms persist, doses are repeated hourly until the score is below 8. Once stable, patients can be assessed every 4–8 h for additional therapy. The symptom‐triggered approach is as efficacious as the fixed‐dose method in managing alcohol withdrawal in terms of efficacy and incidence of adverse events.The advantages of symptom‐triggered therapy are shorter duration of detoxification, lower doses of BZD required, less sedation, and decreased risk of respiratory depression.

Non‐benzodiazepines

Antipsychotic agents

Although they may reduce symptoms of withdrawal, antipsychotics including phenothiazines and butyrophenones, like haloperidol, are associated with higher mortality due to cardiac arrhythmia by prolongation of the QT interval. Furthermore, they lower the seizure threshold. Therefore, antipsychotic agents should be used cautiously in AWS, particularly in its early stage (<48 h) when the seizure risk is high. Nevertheless, they may be considered as adjunctive therapy to benzodiazepines in the late stage of AWS, when agitation, delirium, and hallucinations are not controlled with BZD alone.

Antiepileptic agents

Seven randomized controlled studies, including over 600 patients, have investigated the effectiveness of carbamazepine (CBZ) in comparison with BZD. At daily doses of 800 mg with either a fixed or a tapered regimen over 5–9 d, CBZ was well tolerated and reduced withdrawal symptoms. Nevertheless, due to underenrollment, delayed medication administration, insufficient sample size, and inadequate dosage, the impact of CBZ to prevent seizures or DT is still uncertain and effectiveness compared to BDZ has not been verified. A retrospective analysis of over 700 patients comparing CBZ to valproate (VPA) found VPA to offer some benefits compared to CBZ, such as favorable tolerability and shorter duration of treatment. However, because of the study design and the lack of comparison to BZD, the study did not support implementation into clinical routine. Concerning gabapentin, there were similar results with some effects on mild/moderate withdrawal symptoms but no superiority to BZD.

As levetiracetam (LEV) has no significant affinity to GABAergic and glutamatergic receptors, its mechanism of action in AWS is still unclear. LEV represents a pyrrolidine derivate with binding to the synaptic vesicle protein SV2A, hereby regulating calcium‐dependent neurotransmitter release. Thus, it might reduce excessive neuronal activity and may exert neuroprotective effects. Due to its high tolerability and advantageous pharmacokinetics with lack of drug–drug interactions, LEV appears to be a promising agent in the therapy of AWS. The few available data have shown that the treatment with LEV resulted in a rapid and stable clinical improvement of AWS. Its usefulness in AWS treatment still needs to be investigated.

In summary, besides BZD, anticonvulsants seem to be widely used for the treatment of AWS. Nevertheless, a Cochrane review investigating 56 studies with a total of 4076 participants found no sufficient evidence in favor of any antiepileptic agent for therapy of AWS.

Alpha‐2 agonistic agents

Dexmedetomidine (DEX), a more potent ɑ‐2 agonist than clonidine, decreases sympathetic overdrive and release of norepinephrine. Due to its rapid onset of action and short half‐life, it produces a “cooperative sedation” without necessity for intubation. As ɑ‐2 agonists lack the GABAergic activity to prevent and treat DT or seizures, they can only be used as adjunctive therapy to reduce autonomic hyperactivity that cannot be controlled by BZD alone. Several studies demonstrated a BZD‐sparing effect with significant reduction in BZD requirement.

Anesthetic agents

Propofol

Propofol enhances the inhibitory effects at the GABA‐A receptor and decreases excitatory circuits of the NMDA transmitter system. Due to its strong lipophilic properties, it features a rapid onset of action and is easy to titrate because of the short half‐life. Propofol has general anesthetic effects that often require intubation and mechanical ventilation. Its use is therefore restricted to the intensive care unit making this agent an adjunct therapy for refractory cases of AWS.Its application and experience in AWS is limited to only a few cases and rebound of withdrawal symptoms soon after stopping propofol infusion has been reported.

Barbiturates

Barbiturates are also GABA‐enhancing drugs that work synergistically with BZD featuring a different receptor profile. They can be given orally or intravenously with a loading dose of 100–200 mg/h and have been shown to be as effective as BZD. Unfortunately, barbiturates have a narrow therapeutic index with a long half‐live making titration difficult. They increase the likelihood of respiratory insufficiency and coma so that intubation and mechanical ventilation is often necessary. Because there is no antidote to toxicity, barbiturates are not used frequently in the therapy of AWS.

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Others

Clomethiazole

As the parenteral form of clomethiazole is no longer available, its application is dependent on sufficient alertness and cooperation to enable peroral treatment. For adequate alleviation of delirious symptoms, 200 mg capsules are administered (maximum 24 capsules per day) and doses are repeated every 2–3 h until sufficient calming. As with BZDs, CNS respiratory center depression may emerge, especially in combination with BZDs, whose daily doses should be reduced to 15–20%. Further side effects of clomethiazole are an increased risk of pneumonia due to bronchial mucus accumulation as well as dependence, so that administration should not exceed 10 d. Moreover, clomethiazole is subjected to a pronounced first pass effect by the isoenzyme CYP2E1 which is blocked by ethanol consumption. Accordingly, the combinatory intake of clomethiazole and ethanol should be avoided due to its possible life‐threatening effects.

Gamma‐hydroxybutyric acid (GHB) and Sodium oxybate (SMO)

GHB, admitted to the treatment of narcolepsy, is an endogenous neurotransmitter and a metabolite of GABA. It has a stake in GABA‐dependent neurotransmission, dopamine release, and thereby, it regulates the wake–sleep cycle. GHB acts as a depressant at higher doses and has anxiolytic properties. A Cochrane review shows impact on symptoms of alcohol withdrawal in comparison with placebo, but no superiority to BZDs or clomethiazole in prevention of AWS with a high risk of misuse, abuse, and addiction. SMO is the sodium salt of γ‐hydroxybutyric acid, a naturally occurring short‐chain fatty acid that is structurally similar to GABA. In addition to the activation of the GABA‐A receptor, it has also alcohol mimicking effects due to dopamine release in the CNS. There are some studies showing SMO to be equally effective as BZD in moderate‐to‐severe AWS.When used for a short period, SMO is relatively well tolerated; in long‐term use, there is, as is known for GHB, concern about abuse and dependence based on its euphoric properties.

Baclofen

Baclofen, a GABA‐B receptor agonist and a well‐known muscle relaxant for treatment of spasticity, has similar mechanisms of action and similar effects as SMO. Consistent with preclinical evidence, open‐label reports demonstrated the ability of baclofen to rapidly reduce symptoms of severe AWS and to decrease craving. Due to only a few trials, there is not enough evidence to recommend its use.

Adjunctive Therapeutic Agents

Magnesium

Magnesium is an important cofactor of many enzymes and acts as an inhibitor of neurotransmitter release. Therefore, it may dampen the NMDA‐driven hyperexcitability in AWS by competing with glutamate in its receptor binding site. Furthermore, magnesium impedes the NO synthase and calcium‐dependent channels, lowering action potential firing. As chronic alcohol use is associated with abnormal magnesium metabolism, patients have been given magnesium to treat or prevent AWS. Based on a Cochrane review, there is currently insufficient evidence to support the routine use of magnesium for prophylaxis or treatment of AWS. Nevertheless, as alcohol use and withdrawal are connected with QT interval prolongation and cardiac arrhythmia, laboratory values of magnesium should be determined and deficiencies be balanced.

Thiamine

Wernicke’s encephalopathy (WE) is afflicted with high morbidity and mortality and presents only in rare cases with the classic triad of confusion, ataxia, and ophthalmoplegia. According to the EFNS guideline for diagnosis of WE, two of the following four signs are required: (i) dietary deficiencies, (ii) eye signs, (iii) cerebellar dysfunction, and (iv) either an altered mental state or mild memory impairment. Particularly in severe AWS with predominant symptoms of DT, differentiation from WE is sometimes impossible. Because of its easy and uncomplicated treatment, prevention of WE with parenteral thiamine should be performed in all patients at risk, including those experiencing AWS and prior to any parenteral carbohydrate‐containing fluids. The earlier thiamine supplementation is started, the faster is recovery, regardless of initial clinical presentation.

Conclusions

Alcoholics are a diverse group. They experience different subsets of symptoms, and the disease has different origins and modulating influences for different people. Therefore, to understand the effects of alcoholism, it is important to consider the influence of a wide range of variables on a particular behavior or set of behaviors. The underpinnings of alcohol-induced brain defects are multivariate; to date, the available literature does not support the assertion that any one variable can consistently and completely account for these impairments. Instead, the identification of the most salient variables is a primary focus of current research. In the search for answers, we recommend an integrative approach that recognizes the interconnectivity of the different functional systems to account for the heterogeneity of outcome variables associated with alcoholism-related impairments and recovery of functions. It is helpful to use as many kinds of tools as possible, keeping in mind that specific deficits can be observed only with certain methods, with rigorous paradigms, and with particular groups of people with distinct risk factors. Such confluence of information can provide evidence linking structural damage, functional alterations, and the specific behavioral and neuropsychological effects of alcoholism. These measures also can determine the degree to which abstinence and treatment result in the reversal of atrophy and dysfunction.

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INTRODUCTION-

Alcoholism results from an interplay between genetic and environmental factors, and is linked to brain defects and associated cognitive, emotional, and behavioral impairments. A confluence of findings from neuroimaging, physiological, neuropathological, and neuropsychological studies of alcoholics indicate that the frontal lobes, limbic system, and cerebellum are particularly vulnerable to damage and dysfunction. An integrative approach employing a variety of neuroscientific technologies is essential for recognizing the interconnectivity of the different functional systems affected by alcoholism. In that way, relevant experimental techniques can be applied to assist in determining the degree to which abstinence and treatment contribute to the reversal of atrophy and dysfunction.

The alcohol withdrawal syndrome is a well‐known condition occurring after intentional or unintentional abrupt cessation of heavy/constant drinking in patients suffering from alcohol use disorders (AUDs). AUDs are common in neurological departments with patients admitted for coma, epileptic seizures, dementia, polyneuropathy, and gait disturbances. Nonetheless, diagnosis and treatment are often delayed until dramatic symptoms occur. The purpose of this review is to increase the awareness of the early clinical manifestations of AWS and the appropriate identification and management of this important condition in a neurological setting.

An estimated 76.3 million people worldwide have alcohol use disorders (AUDs), and these account for 1.8 million deaths each year. It is estimated that up to 42% of patients admitted to general hospitals, and one‐third of patients admitted to hospital intensive care units (ICU) have AUD. Alcohol withdrawal syndrome (AWS) is a well‐known condition occurring after intentional or unintentional abrupt cessation of heavy/constant drinking, and it occurs in about 8% of hospitalized AUD inpatients. Severe AWS more than doubles the length of stay and frequently requires treatment at the ICU. A complicated AWS includes epileptic seizures and/or delirium tremens (DT), the occurrence of which may be as high as 15% in AUD patients., Delirious patients show high rates of comorbidities, and their mortality rate is comparable to patients having severe malignant diseases. However, with early detection and appropriate treatment, the expected mortality is in the range of 1% or less.

AUDs are common in patients referred to neurological departments, admitted for coma, epileptic seizures, dementia, polyneuropathy, and gait disturbances. Nonetheless, diagnosis and treatment are often delayed until dramatic symptoms occur. The purpose of this review is to increase the awareness of the early clinical manifestations of AWS and the appropriate identification and management of this important condition in a neurological setting.

Types of Alcohol-Related Neurologic Disorders

There are several neurological diseases that can be caused by alcohol abuse, including fetal alcohol syndrome, dementia, and some symptoms associated with alcohol withdrawal. Alcohol-related neurological diseases include:

• Alcoholic cerebellar degeneration: This is one of the more common forms of cerebellar ataxia, or loss of tissue mass in the brain. The most common symptom associated with cerebellar degeneration involves the loss of the ability to walk over a period of months or years. The condition also affects eye movements, gait or pace, and, rarely, loss of muscle coordination in the upper body. Cerebellar degeneration is caused by malnutrition, but the main cause in most of the Western world is alcohol abuse.
• Alcoholic myopathy: About 33 percent of people struggling with alcohol use disorder develop alcoholic myopathy. This condition involves the breakdown of proximal muscles, which are found in the arms, shoulders, thighs, and upper legs – skeletal muscles found nearest the body’s trunk. Symptoms include:
  • Muscle pain, tenderness, swelling, and weakness, which may appear after a binge or when the person wakes from an alcoholic stupor
  • Cardiomyopathy, or weakening and drooping of the heart muscle so it does not pump blood efficiently
  • Renal damage or failure, when the toxins from muscle breakdown flood the kidneys to the point that the organs can no longer effectively filter them out

Alcoholic neuropathy: Although it is unclear what exactly causes the condition, alcoholic neuropathy is a deadening of nerves throughout the peripheral nervous system, so the person will feel tingling or burning sensations and numbness, and have trouble with basic functions like walking, internal body temperature regulation, and bowel or bladder function. The causes of alcoholic neuropathy likely involve poisoning of the nerves due to high-volume consumption of alcohol and poor nutrition associated with problem drinking. Symptoms of alcoholic neuropathy include:

• Numbness in the extremities, including the arms and legs
• The feeling of “pins and needles” or other abnormal sensations
• Pain or burning in the arms and legs
• Muscle problems, including cramps, weakness, spasms, or aching sensations
• Heat intolerance due to poor regulation, especially after exercise
• Incontinence, trouble urinating, or other bladder problems
• Diarrhea or constipation
• Nausea and vomiting
• Trouble speaking or swallowing
• Unsteady gait, stumbling, or lack of balance

Delirium tremens: When a person suddenly stops drinking after abusing alcohol for many years, they are at risk of developing alcohol withdrawal syndrome, or delirium tremens (DT). The body has developed a physical dependence on alcohol to manage brain function, so when the substance is suddenly removed, life-threatening side effects can develop. These include:

• Severe confusion or delirium
• Physical tremors
• Changes in thinking or memory
• Extreme agitation and irritability
• Intense excitement, fear, or paranoia
• Hallucinations
• Sudden bursts of energy
• Stupor, or being awake but unresponsive
• Seizures or convulsions

Fetal alcohol spectrum disorders: While many alcohol-related neurological conditions occur in people who consume too much alcohol, fetal alcohol spectrum disorders (FASDs) occur in babies or children, and may lead to life-long developmental delays, emotional or behavioral abnormalities, or trouble thinking or learning. Women who consume alcohol while pregnant put their children at risk for FASDs. Types of FASDs include:

• Fetal alcohol syndrome (FAS): Fetal death is the most extreme outcome from a woman struggling with AUD or problem drinking during pregnancy; other forms of FAS include abnormal facial features, growth problems, and developmental differences or disabilities in the central nervous system (CNS). People who live with FAS may have trouble with their memory, attention, communication skills, learning abilities, hearing, and/or vision.
• Alcohol-related neurodevelopmental disorder (ARND): With this disorder, children and adults have intellectual disabilities, behavioral struggles, and trouble learning.
• Alcohol-related birth defects: These involve physical problems, including damage to the heart, kidneys, hearing, or bones during fetal development.

Wernicke-Korsakoff syndrome: This condition is a combination of two forms of brain disease, Wernicke’s encephalopathy and Korsakoff syndrome. Both of these conditions are triggered by a loss of thiamine, or vitamin B12, which is most often caused by problem drinking. When a person struggles with heavy drinking or alcohol use disorder, they are more likely to drink alcohol than eat regular meals, and the body will struggle to absorb nutrients from food over time.Symptoms of Wernicke’s encephalopathy include:

• Reduced mental activity
• Confusion
• Ataxia, starting with muscle tremors in the legs and leading to loss of muscle control overall
• Abnormal eye movements
• Double vision
• Eyelid drooping
• Withdrawal symptoms if the person stops drinking

Korsakoff syndrome’s symptoms include:

• Trouble forming new memories
• Trouble remembering old memories, leading to fabrication of events or people
• Other forms of severe memory loss
• Hallucinations, especially visual or auditory

In some cases, one or both syndromes will clear up on their own; however, in 25 percent of Wernicke-Korsakoff syndrome cases, the problem does not stop on its own and is likely to get worse. This is especially true if the person does not stop drinking alcohol and get treatment.

Pathophysiology

Ethanol is a central nervous system depressant that produces euphoria and behavioral excitation at low blood concentrations due to increased glutamate binding to N‐methyl‐D‐aspartate (NMDA) receptors; at higher concentrations, it leads to acute intoxication by potentiation of the gamma‐aminobutyric acid (GABA) effects, particularly in receptors with delta subunits. The local distribution of these subunits explains why the cerebellum, cortical areas, thalamic relay circuitry, and brainstem are the main networks that mediate the intoxicating effects of alcohol. Prolonged alcohol use leads to the development of tolerance and physical dependence, which may result from compensatory functional changes by downregulation of GABA receptors and increased expression of NMDA receptors with production of more glutamate to maintain central nervous system (CNS) transmitter homeostasis.

Abrupt cessation of chronic alcohol consumption unmasks these changes with a glutamate‐mediated CNS excitation resulting in autonomic overactivity and neuropsychiatric complications such as delirium and seizures. The latter are usually of generalized tonic–clonic type and are mediated largely in the brainstem by abrogation of the tonic inhibitory effect of the GABAergic delta subunits. Therefore, the trigger zone of these seizures is distinct from that believed to be responsible for seizures in the context of epilepsy, and this may explain why epileptiform activity is rarely observed in the EEG after alcohol withdrawal seizures. As upregulation of NMDA receptors as well as reduced GABA‐A receptor inhibition largely explain the clinical symptoms, the therapeutic approach to AWS mainly targets these mechanisms. Dopamine is another neurotransmitter involved in alcohol withdrawal states. During alcohol use, increase in dopamine positively influences the reward system thereby maintaining abuse. In withdrawal, increase in dopamine levels contributes to the clinical manifestations of autonomic hyperarousal and hallucinations. Moreover, polymorphisms in the dopamine receptor 2 gene seem to influence not only AUD but also the clinical manifestation of alcohol withdrawal symptoms. In combination with increased glutamate and norepinephrine, it may also cause the elongation of the QT interval in people who have active epilepsy; this can increase the risk of sudden unexpected death in epilepsy (SUDEP).Another excitotoxic compound that is increased in AUD is homocysteine. During active drinking, there is an increase in homocysteine through stimulation of the NMDA receptors. In withdrawal, excitotoxicity is induced by further raise in homocysteine via rebound activation of glutamatergic neurotransmission.

Clinical spectrum

AWS represents a group of symptoms that usually arise 1–3 d after the last drink. Sometimes, the symptoms are already present when the alcohol blood level is above 0 (0.5‰ or even more). The Diagnostic and Statistical Manual of Mental Disorders (DSM‐5) outlines diagnostic criteria for AWS using two main components so that the AWS is diagnosed when the following two conditions are met:

1. A clear evidence of cessation or reduction in heavy and prolonged alcohol use.
2. The symptoms of withdrawal are not accounted for by a medical or another mental or behavioral disorder.

Autonomic symptoms	Motor symptoms	Awareness symptoms	Psychiatric symptoms
Tachycardia	Hand tremor	Insomnia	Illusions
Tachypnea	Tremulousness of body	Agitation	Delusions
Dilated pupils	Seizures	Irritability	Hallucinations
Elevated blood pressure	Ataxia	Delirium	Paranoid ideas
Elevated body temperature	Gait disturbances	Disorientation	Anxiety
Diaphoresis	Hyper‐reflexia		Affective instability
Nausea/vomiting	Dysarthria		Combativeness
Diarrhea			Disinhibition

The alcohol withdrawal syndrome is a dynamic and complex process. For this reason, there have been many attempts to classify symptoms of AWS either by severity or time of onset to facilitate prediction and outcome. In early stages, symptoms usually are restricted to autonomic presentations, tremor, hyperactivity, insomnia, and headache. In minor withdrawal, patients always have intact orientation and are fully conscious. Symptoms start around 6 h after cessation or decrease in intake and last up to 4–48 h (early withdrawal, Hallucinations of visual, tactile or auditory qualities, and illusions while conscious are symptoms of moderate withdrawal. They can last up to 6 d. The appearance of acute symptomatic seizures may emerge 6–48 h after the last drink. Delirium tremens (DT, onset 48–72 h after cessation of drinking) represents characteristics of severe withdrawal that may last for up to 2 weeks (late withdrawal)

The alcohol withdrawal seizure is a symptom occurring primarily during the early phase of withdrawal and is characterized by reduction in the seizure threshold. More than 90% of acute symptomatic seizures emerge within 48 h of cessation of prolonged drinking. Seizures frequently occur in the absence of other signs of the AWS. More than half of the individuals present with repeated seizures, and in up to 5%, they may progress to status epilepticus. More than 50% of withdrawal seizures are associated with concurrent risk factors such as prior epilepsy, structural brain lesions, or use of other drugs. It is remarkable that the development of acute symptomatic seizures during an alcohol withdrawal episode is associated with a fourfold increase in the mortality rate that is due to complications of severe AUD rather than a direct effect of seizures. The appearance of a withdrawal seizure represents a strong risk factor for progression into a severe withdrawal state with following development of DT in up to 30% of cases. Unprovoked seizures occurring later than 48 h after the last drink suggest other causes such as head trauma or combined drug withdrawal effects.

Delirium is a clinical syndrome of acute onset characterized by a global confusional state, perceptual abnormalities, and somatic symptoms of vegetative or central nervous presentation. Hallucinosis represents a unique form of withdrawal‐related psychosis which can begin even while the person is continuing to use alcohol or after cessation of drinking. The sensorium is clear in the beginning, but it often evolves into the syndrome of DT, a specific type of delirium typically associated with psychomotor agitation (hyperactive delirium) which emerges during the late withdrawal phase. Delirium can also manifest as a hypoactive state with decreased arousal and psychomotor activity, which is associated with a worse prognosis, delayed diagnosis and treatment as well as later complications. In cases of hypoactive delirium, comorbid or other medical illnesses must be ruled out. This is especially important in patients who have not had a previous history of DT.

Hyponatremia	Due to poor oral intake, dehydration, and uremia; frequently presenting as hypoactive delirium
Hepatic encephalopathy	Jaundice, hematemesis, melena, icterus, flapping tremor, ascites, sleep–wake reversal
Pneumonia	Fever, cough, low arterial blood oxygen saturation, delirium before cessation of alcohol use
Encephalitis/Meningitis	Fever, meningeal signs, and focal neurological deficits; MRI/CSF abnormalities
Head injury	Being found unconscious, ear or nose bleeding, pinpoint pupils, focal neurological deficits
Thyrotoxicosis	History of thyroid illness; thyromegaly, exophthalmos, lagophthalmos
Lithium intoxication	History of psychiatric illness, drug overuse, diarrhea, fever, use of NSAID or diuretics
Atropine/Tricyclic intoxication	Fever, hot dry skin, dilated pupils
Psychosis	Hallucinations/delusions of long‐standing duration, absence of clouding of sensorium
Antidepressant intoxication	Use of SSRI; diarrhea, myoclonus, jitteriness, seizures, altered sensorium
Subacute encephalopathy with seizures in AUD	Several days after alcohol cessation; complex/simple partial seizures with reversible motor deficits; in EEG focal slowing, periodic lateralized discharges; MRI with reversible T2w flair hyperintensities

In summary, physical examination and investigations should be directed toward detecting signs of intoxication, seizures, hallucinations, and delirium tremens as well as Wernicke’s encephalopathy (one or more symptoms of ataxia, amnesia, and ophthalmoplegia). Apart from neuropsychiatric symptoms, physical injury or medical problems including aspiration pneumonia, dehydration, and electrolyte imbalance should be taken into account.

Biomarkers

In several studies, possible predictors for the development of a severe AWS have been investigated. Medical history and laboratory biomarkers are the two most important methods for the identification of patients at high risk. It appears that the most robust predictor for an incident occurrence of DT or seizures is a history of a similar event.Clinical findings such as elevated heart rate, systolic blood pressure, and temperature are all easily verifiable in the initial patient assessment, although their predictive value to identify patients with AWS who are more likely to develop DT is not high. In a patient with impaired consciousness, laboratory markers represent helpful tools to confirm the suspected clinical diagnosis of an AUD.

Markers useful in the emergency setting

The quantitative, measurable detection of drinking is important for the successful treatment of AUD. Therefore, the importance of direct and indirect alcohol markers to evaluate consumption in the acute clinical setting is increasingly recognized. he detection of ethanol itself in different specimens is still a common diagnostic tool to prove alcohol consumption. Alcohol ingestion can be measured using a breath test. Although ethanol is rapidly eliminated from the circulation, the time for detection by breath analysis is dependent on the amount of intake as ethanol depletes according to a linear reduction at about 0,15‰/1 h. Alcohol use can alternatively be detected by direct measurement of ethanol in blood or urine. The time course of the ethanol concentration in the blood after the ingestion of an alcoholic beverage is controlled by its pharmacokinetics that represents an interplay between the kinetics of absorption, distribution, and elimination and is thus important in determining the pharmacodynamic responses to alcohol. There is a large degree of variability in alcohol metabolism as a result of both genetic and environmental factors.

Ethanol	BreathBloodUrine	<6 h	5–24 hdepletion 0,15‰/1 h	~ 90%/~ 95%	Conversion factor breath alcohol:blood alcohol 1:2100 within 2–5 h after the last drink
Hypokalemia	Blood	<6 h	Days to weeks	~ 47%/~ 90%	Serum levels <2,5 mmol/L indicate severe AUD
Thrombocytopenia	Blood	<6 h	7–12 d	~ 69%/~ 75%	High NPV, low PPV; rebound thrombocytosis after cessation of alcohol abuse
Mean corpuscular volume	Blood	<6 h	4 mo	~ 80%/~ 60%	Dose‐dependent increase
γ‐glutamyltransferase	Blood	<6 h	2–8 wk	~ 80%/~ 65%	Severe AUD with liver damage
Ratio AST/ALT >2	Blood	<6 h	AST 18 hALT 36 h	~ 50%/~ 80%	Severe AUD, marker of liver damage

Apart from ethanol itself, indirect markers of AUD are widely available and mostly part of routine laboratory testing. Severe AWS involves changes in electrolytes, especially potassium that is due to increased catecholamine activity with activation of the sodium–potassium ATPase pump and elevated vasopressin. Hypokalemia is not specific for alcohol consumption but is frequently reported to be associated with DT or seizures. The same applies to thrombocytopenia (with high negative predictive value) that additionally is predictive of an incident occurrence of DT and seizures. More indirect markers, such as AST, ALT, γGT, and MCV, are widely available and relatively inexpensive, but their predictive value is restricted because of low specificity. The interpretation of elevated values has to take into account other influencing factors including gender, age, comorbid disorders, and medication that also may increase these markers.

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Additional markers to detect AUD

Further biomarkers for non‐emergency cases or in the event of forensic questions are listed in Table . Carbohydrate‐deficient transferrin (CDT) is the most available and studied biomarker and has a high specificity for severe AUD. CDT values are not markedly influenced by medications except by immunosuppressants. The main disadvantage is the relatively low sensitivity making this parameter unsuitable as a screening tool. As CDT, γGT, and MCV are connected with AUD by different pathophysiological mechanisms, a combination of these parameters will further improve their diagnostic value.

Carbohydrate‐deficient transferrin	Blood	>6 h	2–4 wk	~ 98%/~ 70%	Severe AUD	24, 37
Ratio γGT:CDT	Blood	>6 h	2–3 wk	~ 92%/~ 84%	Severe AUD	24, 32
Ethylglucuronid	Bloodurinehair	>6 h	8 h20–80 h3 mo	~ 99%/~ 89%	Values dependent on creatinine clearance	31, 37
Ethylsulfate	Bloodurine	>6 h	8 h36–78 h	~ 99%/~ 89%	Values dependent on creatinine clearance	31, 37
Phosphatidylethanol	Blood	>6 h	4 wk	~ 99%/~ 98%	Detection also available for dry blood spots	27, 31, 42, 53
Fatty acid ethyl esters	Bloodhair	>6 h	24 h3 mo	~ 97%/~ 77%	Combined measurement of ethylglucuronide and fatty acid ethyl esters in hair increases accuracy of interpretation	27, 31, 44, 54
5‐hydroxytryptophol:5‐hydroxyindole‐3‐acetic acid	Urine	>6 h	24 h	~ 99%/~ 77%	Ratio >20 marker for recent alcohol intake	47, 48
Whole blood acetaldehyde	Blood	>6 h	4 wk	~ 93%/~ 78%	False‐positive results in diabetics	55
Total sialic acid	Blood	>6 h	Several weeks	~ 95%/~ 81%	Glycoconjugate metabolite	37, 44, 56
Homocysteine	Blood	>6 h	Several weeks	~ 61%/~ 72%	Cutoff ~24 μmol

Apart from indirect markers for AUD, more specific alternatives focus on metabolic markers comprising direct products of alcohol degradation, that is, phosphatidylethanol (Peth), ethylglucuronide (EtG), ethylsulfate (EtS), and fatty acid ethyl esters (FAEE). Their presence is closely connected to alcohol consumption, and the well‐known CDT as well as sialic acid and EtG are the result of alcohol‐induced glycoconjugate metabolites. The highest sensitivity of up to 99% was observed for Peth that showed a rapid decrease at the beginning of withdrawal, a slow decline after the first few days, and persistence at low levels beyond 19d of abstinence. Apart from biomarkers detected in blood and urine samples, saliva is a promising and easy accessible material to detect glycomarkers of oxidative stress, but the reproducibility and validity in Peth has to be proven for clinical routine application. As an antibody based flash test is available for detection of EtG in urine with good sensitivity and specificity, this parameter is the most promising one to be integrated in routine laboratory settings and in screening of patients at risk to develop AWS.

As long as ethanol is metabolized, the metabolism of serotonin is shifted from formation of 5‐hydroxyindole‐3‐acetic acid (5‐HIAA) toward 5‐hydroxytryptophol (5‐HTOL). The 5‐HTOL/5‐HIAA ratio increases appreciably in urine after alcohol intake and is a promising marker for recent alcohol intake with a short window of detection. Until now, it has not found its way into clinical routine because of costly detection assays.

Recent investigations pointed out that homocysteine levels on admission might be a useful screening method for the risk of seizures in AWS, particular in combination with CDT. Several days after alcohol abstinence, homocysteine plasma levels decrease to normal. Homocysteine levels are influenced by nutritional status, gender, and age. Its metabolism is dependent on the enzyme 5,10‐methylenetetrahydrofolate reductase (MTHFR). The single‐nucleotide polymorphism MTHFR C677T elevates plasma homocysteine levels. Lutz et al. investigated two groups of patients with AWS and found this polymorphism to be related to higher occurrence of withdrawal seizures in the Western European population.

ALCOHOL AT A NEUROTRANSMITTER LEVEL

Alcohol’s central nervous system (CNS) effects are mediated through actions on a variety of neurotransmitters. There is a complex interplay between excitatory and inhibitory systems . The numerous transmitters involved in alcohol’s action explain its diverse effects and the large number of drug interactions with both prescribed and illicit drugs.

Alcohol and neurotransmitters

• Dopamine: alcohol increases dopamine use in the nucleus accumbens, mediating its pleasurable effects via the common reward pathway of the mesolimbic system

• Noradrenaline: alcohol release of noradrenaline (norepinephrine) contributes to the enlivening and activating “party” effects of alcohol

• Endogenous opioids: Alcohol’s analgesic, pleasure, and stress reducing functions are opioid related

• GABA: Alcohol can potentiate GABA (γ aminobutyric acid) activity through certain subunits of the GABA A receptor. This accounts for alcohol’s anxiolytic and ataxic actions, and partially for amnesia and sedation.

• Glutamate: Alcohol acts to block the excitatory NMDA (N-methyl-d-aspartate) receptor, opposing glutamate causing amnesia and other cerebral depressant effects

• Serotonin: Alcohol’s stimulation of 5HT3 (5-hydroxytryptamine 3) provides the nausea associated with alcohol use. Serotonin may also be linked to the pleasurable effects of alcohol and differing brain serotonin levels may distinguish between anxious and aggressive alcohol users

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Differential Diagnosis

• Sacrococcygeal teratomas
• Benign teratomas
• Subcutaneous lipomas
• Lymphangiomas
• Cystic teratomas
• Spinal epidural abscess
• Spinal cord masses
• Pilonidal cyst
• Inclusion dermoid
• Caudal regression syndrome

Assessment

Initial assessment of the newborn is extremely important, preferably both by a pediatrician and a neurosurgeon. Examination of head and neck involves the assessment of head size, shape, skull bones, and openness of fontanelles, lacunar skull defects and the size of the posterior fossa. Examination of the back needs assessment of the neural placode, level of the lesion, condition of the skin, extent of skin defect and associated deformities. Examination of the lower limbs for detection of the deformities of the foot and abnormalities of long bones is important. A detailed neurological examination is necessary to assess the level of motor weakness, sensory level and sphincter dysfunction.

Prenatal diagnosis

Prenatal screening for neurological abnormalities is based on ultrasound performed routinely or oriented by maternal Alpha Feto Protein (AFP) screening. It should be performed around 12, 22 and 32 weeks. Maternal serum screening can detect up to 80% cases of spina bifida and 90% cases of anencephaly. Sonography may identify up to 90% cases of myelomeningoceles. Over the last few decades, the diffusion of routine ultrasound has changed the spectrum of the neonatal neurological malformations. Gross lethal abnormalities nearly always result in termination of pregnancy, depending on the regional law system. A growing number of more subtle abnormalities including midline or posterior fossa abnormalities are being discovered. But their postnatal outcome cannot always be predicted accurately, despite the use of fetal MRI. Maternal serum screening for chromosomal abnormalities is also increasingly used. Only in select situations, amniocentesis is contributory.

Complications

Bladder dysfunction: Most patients with myelomeningocele have some degree of bladder incontinence. Preventive goals are directed toward preventing infection with the implementation of bladder drainage utilizing intermittent catheterization or indwelling catheters. Bladder stimulation has shown to improve bladder emptying and reduce infection.  

Bowel dysfunction: Myelomeningocele is associated with anal sphincter dysfunction that results in bowel incontinence. Assisted bowel emptying reduces barriers associated with social activities, including attending school and personal relationships.

Immobility: Most myelomeningocele patients have significant weakness, which results in severe ambulation deficits or paraplegia. Bracing using external orthosis can help to maximize their mobility and ensure a near-normal developmental progression. In children over 1-year-old, utilizing a standing frame can reduce the risk of osteoporosis and the formation of contractures in lower extremities. A wheelchair can provide mobility for older children and adults.

Infections: Due to a neurogenic bladder, many have urine colonization and infections. Shunts are also prone to infections. When shunts are placed, infections can occur superficially at the skin or intraabdominal, as many of these patients have multiple abdominal procedures.

Management

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Management of these children needs multidisciplinary approaches. Complete clinical evaluation and appropriate investigations are necessary. Parents need to be counseled and informed regarding the immediate as well as long-term management strategy.

Surgical treatment

The aim of surgery is to free the placode from the surrounding abnormal skin and reposition into the spinal canal with reconstruction of the dura and coverings to prevent CSF leak and infection. The surgical technique depends on the size and the level of the lesion. The help of pediatric, orthopedic and plastic surgeons may be necessary. Several attempts for maternal fetal surgeries to improve their outcomes have been made. The role of fetal surgery for myelomeningocele is yet to be proven.

Timing of intervention primarily depends on the clinical condition of the child and the impending risks. Surgery need not be done as a compelling emergency but should be undertaken as soon as it is practical. In case of suspected meningitis or CSF infection or colonization of the wound, prophylactic antibiotics and anticonvulsants form the initial treatment. Child is nursed in an incubator; routine blood counts and serum electrolytes are monitored. Blood grouping and cross matching is done for possible transfusion. Careful assessment of body weight is essential for intraoperative management. The newborn child with myelomeningocele should have saline dressings. It is essential not to use corrosive agents, spirit or antiseptics indiscriminately over the open defects to avoid damage to the underlying exposed neural tissue.

Surgical technique

To obtain successful repair, it is essential to study the surface anatomy and its relationships to the surrounding structures. At the apex of the myelomeningocele usually, the flat neural placode is located and from its edge the remnants of the arachnoid membrane get attached at the nerve root entry zone. From this junction, the nerve roots emerge and exit through neural foramina located ventrally. They are seen through the transparent arachnoid membrane which is fused with the skin at the lateral edges of the lesion. The dura matter which is defective posteriorly is loosely adherent to the underlying soft tissue of the back and densely adherent to the bony structures underneath. Rostrally, the dura forms tube and the neural placode continues into it, which leads to functional spinal cord.

Medical Management

Medical management of the newly born child with Spina Bifida varies according to the severity of their condition. Those with Spina Bifida Occulta do not ususally require any specific treatment. Some people with Spina Bifida Occulta do not exhibit any symptoms and may only discover they have the condition when they are older after having an XRAY. Children born with myelocoele or myelomeningocoele will require surgery normally within 2-3 days of birth in order to close the gap in the spine and return the spinal cord and nerves to their original place within the spinal column. This aims to prevent infection and further damage to the exposed spinal cord and spinal nerves. Following surgery, the child will be monitored closely for signs of common pos-operative problems associated with this type of surgery, namely hydrocephalus and leaking of cerebrospinal fluid

As the infant gets older, management of incontinence will be an important role of the medical team. Effective management strategies include the use of Clean Intermittent Catheterisation (CIC) and certain drugs which can increase the storage volume of the bladder . Children can also develop constipation due to lack of bowel movements and will require the development of a bowel programme which may involve assisted evacuation of stools. However, this will be based on an individualised assessment of the child and may involve educating the family in order to ensure the programme is effectively integrated into the child’s daily routine Effective strategies in managing incontinence in children with spina bifida are extremely important in allowing them to socially integrate themselves as they get older and attend school . The management of spina bifida varies depending on the degree the individual is affected with the disease. with acquired brain injury. A cross-sectional study (August 2020) by a multidisciplinary team describing health issues and living conditions in a cohort of adults living with Spina bifida suggests the presence of a higher prevalence of urinary and faecal incontinence, pain, and overweight in adults with Spina Bifida. Persons with the condition greater than 46 years had less complicated medical conditions, better physical and cognitive functions, and higher education, independent living, and participation in society, whereas individuals < 46 years had more secondary conditions such as hydrocephalus, Chiari II malformation, tethered cord symptoms, and latex allergy. 

pina Bifida Occulta:

• There is generally no medical treatment required

Spina Bifida Meningocele:

• Surgery is often performed early after birth, but the severity of deficits after surgery depends on if there is neural tissue in the sac.  Further treatment is similar to the management listed below for myelomeningocele.

Spina Bifida Myelomeningocele:

• Generally, surgery follows within the first few days of life to close the spinal cord defect. It is crucial during this time period prior to surgery to protect the nerves that are exposed in the protruding sac. It is also important to prevent infection and additional trauma to the exposed tissues.
  
• Additional surgeries may be required to manage other problems in the feet, hips, or spine. The individuals with hydrocephalus will also require subsequent surgeries due to the shunt needing to be replaced.
  
• The level of malformation of the spinal cord and subsequent neurological defects will influence the individual’s ability to ambulate. Assistive devices may be necessary to aid the individual around the community.
  
• Due to the bowel and bladder problems that are often caused by the neural tube defect, a bowel and bladder program may be necessary. This may include catheterization or a strict bowel and bladder regimen to remain regular.
  
• The MOMS study is a trial that was done to look at the effectiveness of having fetal surgery to fix the malformation of the fetus’s spine prior to birth in comparison to waiting until after the child is born to have the surgery.  The idea behind it was that neurological function tends to decrease as pregnancy progresses, so by performing the surgery in utero the baby would not be exposed to such extensive neurological deficits as it would if the surgery was performed after birth.  However, there is a safety concern for both the mother and the fetus when this fetal surgery is performed.  The success of the MOMS trial has now made fetal surgery a treatment option in some cases.

Neurogenic bladder is a common complication for people with spina bifida. It is normally treated with pharmaceuticals and intermittent catheterization; however for some patients this treatment does not suffice. New research suggests the idea of tissue engineering and neuromodulation.

Tissue engineering is used to generate new tissue to augment the bladder. Two different theories are utilized: unseeded and seeded. Unseeded “involves the incorporation of a scaffold material (synthetic or biologic) into the host organ, which is used as a template for the ingrowth of native cells that then initiate the regenerative process.” Seeded technology is similar to unseeded; however, it adds “cultured cells to the scaffold prior to implantation into the host.”

Neuromodulation modifies the innervation of the bladder so it can potentially function in a normal manner. Neuromodulation includes “non-operative measures such as transurethral electrical bladder stimulation, minimally invasive procedures such as implantation of a sacral neuromodulation pacemaker device, as well as operative measures that reconfigure sacral nerve root anatomy.”

Researchers are still in the early stages of development for this treatment option, however with advancements in technology is could prove to be a promising option for patients with spina bifida.

Physical Therapy Management

The role of the physiotherapist in the early management of children with spina bifida is extremely important as it helps the child to develop an efficient and purposeful movement that can be incorporated into everyday tasks. By optimising and maintaining mobility, this can eventually help children to become more independent as they get older. The physiotherapist will perform an initial assessment of the infants muscle strength and range of movement available at certain joints. This will allow the physiotherapist to determine which muscles are working properly and which ones are weak. This will give them a baseline measurement to use as a comparison as the child grows. This will also allow the physiotherapist to consider what problems the infant may have as they get older and what type of assistive devices or splints they may require when they begin to mobilise. The physiotherapist will specifically be involved in:

Joint Range of Motion

• In the early stages following surgery, the physiotherapist will begin passive range of motion exercises on the infant’s legs  . This will normally be performed 2-3 times a day. They will also demonstrate this technique to parents or carers so that they may continue to do these exercises as a home exercise programme when the infant is discharged. They may progress these exercises to mimic more functional movements which are related to normal everyday movement patterns. For example, whilst bending the left knee and hip, the right side will be kept straight as would happen in a normal walking pattern. These gentle exercises will help to maintain and may help increase the available range of motion available in joints where the movement restriction is mild. In those who have more pronounced restriction, the physiotherapist may advise that the number of exercise repetitions is increased and the movement is held for longer. The ultimate aim of range of motion exercises is to enable the child to learn and perform them independently as they grow up. It is important for the child to continue with these exercises because when they are moving independently, the functioning muscles may not be working through full range of motion. Passive range of motion exercises will therefore help to maintain flexibility and avoid the development of muscle tightenings known as contractures .

Muscle strength

• Altered muscle tone is a common symptom of spina bifida,therefore, physiotherapists use resistance training in order to strengthen these muscles that have been weakened. This is normally introduced when the infant is old enough to self mobilise. The physiotherapist can develop a programme of strength and endurance training which has been seen to improve functional abilities in children with spina bifida. These training programmes may involve a variety of exercises for the upper and lower limbs, as well as muscles of the trunk and can help improve upper limb strength and cardiovascular fitness .

Positioning and Handling

• Following the first few days after surgery, the infant will normally be placed inside or stomach lying. As the infant begins to stabilise and recover from surgery, the physiotherapist will offer advice as to how to hold the newborn child safely. This is incredibly important as the infant will have undergone major surgery which requires careful handling and positioning at all times. It may be advised that parents or carers hold the child underneath the stomach and across their forearm due to the surgical wound that will be present on the infant’s back. This handling technique may be used when sitting or walking around. When advised, parents or carers may take the infant for a walk around the hospital resting over the shoulder. This can encourage the child to begin to lift his or her head and begin to develop head and neck control .

Mobility and Ambulation

• Mobility problems in children with spina bifida can vary according to the level of the spine that has been affected during development . A child with a lesion in the lower back (Lumbar or Sacral levels), is more likely to be able to independently mobilise than one with a lesion in the upper thoracic spine. This can determine whether the child will require a wheelchair, orthotics or assistive devices.
• Parents and carers are often discouraged from using assistive devices such as infant walkers, jumpers and bouncer chairs as these can delay motor development. Infants require active movement and sensory information from the surrounding environment in order to learn how to move efficiently against gravity and maintain erect sitting and standing postures. This is no different for children with Spina Bifida. Infants with spina bifida benefit from movements that challenge control of the head, neck and torso, rather than the use of passive sitting devices or chairs. Active movement allows them to participate in the learning process. For example, rather than using a walker, parents are advised to physically hold their child in the standing position with as little support as possible to promote the necessary control of the legs and torso. This also allows the child to receive feedback from the floor and the surrounding environment .
• As the child begins to mobilise and ambulate more independently, he or she may be fitted for braces or splints to address any deformities caused by muscle imbalance or joint limitations. Orthoses such as braces and splints are supportive devices aimed at optimising existing muscle function and giving support where the child requires it. The earlier these are fitted and provided, the earlier the child will be prepared for the upright position required of standing and walking. It therefore also enhances normal developmental progression and will eventually help the child take part in normal activities of their age group . Children with Spina Bifida lesions in the upper thoracic regions of the spine may require bracing or splinting of the whole leg up to the level of the hip and chest. This is known as a Hip-Knee-Ankle-Foot Orthoses (HKAFO). Others may require orthotics aimed at stabilising the knee, ankle and foot. These are known as Knee-Ankle-Foot orthoses (KAFO) and Ankle-Foot Orthoses (AFO) Reciprocal Gait Orthoses (RGO) may be also provided in order to promote a normal rhythmic walking pattern in the child Children may require the additional use of crutches along with orthoses in order to take some stress off the legs. and standing frames are also used to help children with more severe limitations bear weight through their legs and maintain a full range of motion at all lower limb joints . Furthermore, some children may require casting as a way of treating and preventing contractures. Casting aims to develop a gradual increase in the range of motion available at a certain joint and is a very effective method of improving range of motion at tight joints without the use of surgery . Other children may benefit from the use of a wheelchair, as it can give them more freedom of movement if their walking is limited and strenuous. This can be alternated with the use of orthosis for shorter distances. A wheelchair can also help children keep pace with other able-bodied people, and enable them to participate in recreational activities at school .

Parent/carer education

• Physiotherapy management will eventually be handed over to the parents or carers of the infant. Initially they will be encouraged to observe the physiotherapist carrying out ra ange of motion exercises and handling and positioning strategies before being asked to duplicate these treatments independently. Following these teaching sessions, certain roles will then be handed over to the parents and carers. Following discharge home and as the child begins to mobilise more independently the parents and carers should actively become involved in assessing their child’s progression through observations at home when playing, sitting, crawling etc. This can help with early identification of any differences in the child’s movements or sitting postures between the home and the hospital. It may also allow other possible problems to be identified early on so that a management strategy may be developed. This is essential particularly later on when the child becomes more medically stable, as they will not receive as much medical input and interaction as when the child was a new orn infant in hospital
• The physiotherapist, along with other members of the healthcare team, will be able to offer advice and help parents and carers build confidence in their ability to manage their child’s daily routine

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INTRODUCTION-

Spinal cord development occurs through three consecutive periods of gastrulation, primary nerulation and secondary neurulation. Aberration in these stages causes abnormalities of the spine and spinal cord, collectively referred as spinal dysraphism. They can be broadly classified as anomalies of gastrulation (disorders of notochord formation and of integration); anomalies of primary neurulation (premature dysjunction and nondysjunction); combined anomalies of gastrulation and primary neurulation and anomalies of secondary neurulation. Correlation with clinical and embryological data and common imaging findings provides an organized approach in their diagnosis.

Spinal dysraphism refers to the congenital abnormalities of the spine and spinal cord. Clinico-radiological classification of spinal dysraphism has been well established and widely followed. The objective of this article is to illustrate the common magnetic resonance imaging (MRI) findings of various spinal dysraphisms based on embryological events

Spinal dysraphism is an umbrella term that describes a number of conditions present at birth that affect the spine, spinal cord, or nerve roots.

• Spine: the bony structure also known as the spinal column. Made up of individual vertebrae (bones), the spine protects the spinal cord.
• Spinal cord: the bundle of nerves and other tissue that connects brain and body. Inside the spine, the spinal cord is also protected by a series of membranes (coverings). The spinal cord relays sensory information from the body to the brain, and movement instructions from the brain to the body.
• Nerve roots: nerves that branch off the spinal cord to reach the rest of the body.

All forms of spinal dysraphism result from an event very early in an embryo’s development. In about the third week of development, a sheet of cells called the neural plate folds up to form a structure called the neural tube. The top of the neural tube develops into the brain, and the rest of the neural tube develops into the spine and spinal cord. Spinal dysraphism results when a section of the neural tube that will become the spine and spinal cord does not close completely.

Types of spinal dysraphism include myelomeningocele (also known as spina bifida aperta or open spina bifida), spina bifida occulta, split cord malformation (diastematomyelia), spinal cord lipoma (lipomyelomeningocele), dermal sinus tract, tight filum terminale, and tethered spinal cord. A person with spinal dysraphism may have more than one type.

• Myelomeningocele, or spina bifida aperta: a condition in which the spinal cord and its membranes are not contained within the spinal column, but protrude into a sac outside the body
• Spina bifida occulta: a condition in which one or more vertebrae (bones of the spinal column) has a slight defect, but the spinal cord and its membranes are not affected
• Split cord malformation (diastematomyelia): a complex type of spinal dysraphism in which the spinal cord splits lengthwise into two distinct cords. Often, the split is caused by a thin segment of bone or cartilage that protrudes from the spinal column into the spinal cord space. Sometimes there is only one protective membrane or sleeve (the dura) around both parts of the spinal cord. Sometimes each part has its own dural sleeve.

• Spinal cord lipoma (lipomyelomeningocele): a condition in which an abnormal growth of fat attaches to the spinal cord, its membranes, and the space outside the spinal canal.   Dermal sinus tract : a channel in the skin that may reach all the way to the spinal cord. Dermal sinus tracts are often associated with tumors in the space around the spinal cord called dermoids or epidermoids. Benign (non-cancerous) tumors and cysts can be found along with any type of spinal dysraphism. Tumor types include lipoma (a fatty tumor), dermoid(a tumor containing tissue of hair, bone, or cartilage), and epidermoid(a tumor of skin layers). These tumors are not cancerous and will not spread. However, they may compress the spinal cord or
• tethered spinal cord.Tethered cord: a condition that may occur as a result of any spinal dysraphism, or as a result of other conditions (e.g. tumor, infection, or scar tissue formation). In this condition, the spinal cord is restricted at its base and cannot move freely in the spinal column. The resulting “stretch” on the spinal cord tissue can cause damage to the spinal cord leading to neurological problems (weakness, sensory loss), urological problems (incontinence), orthopedic problems (scoliosis and foot, ankle or leg deformities) and pain (see below). Accumulations of fluid in the spinal cord can also occur.

CLASSIFICATION-

Spinal dysraphism can be broadly divided into two different clinicoradiological entities ^8,9:

• open spinal dysraphism (formerly spina bifida aperta or cystica): occurs when the cord and its covering communicate with the outside; no skin or tissues cover the sac
  • myelomeningocele (98% of open spinal dysraphism)
  • myelocele
  • hemimyelomeningocele
  • hemimyelocele
• closed spinal dysraphism (formerly spina bifida occulta): occurs when the cord is covered by other normal mesenchymal elements
  • with subcutaneous mass
    • lipoma with dural defect
      • lipomyelomeningocele
      • lipomyelocele
    • terminal myelocystocele
    • meningocele
    • limited dorsal myeloschisis
  • without subcutaneous mass
    • posterior spina bifida (isolated defect of the posterior neural arch of vertebra)
    • intradural lipoma
    • filar lipoma
    • tight filum terminale
    • persistent terminal ventricle
    • disorders of midline notochordal integration
      • dorsal dermal sinus
      • dorsal enteric fistula
      • neurenteric cyst ^5,6
      • split cord malformations
        • diastematomyelia
        • diplomyelia
    • disorders of notochordal formation
      • caudal regression syndrome
        • Type 1
        • Type 2
      • segmental spinal dysgenesis

CAUSE AND RISK FACTOR-

The causes of spinal dysraphism are not yet completely understood. Genetic and environmental factors both seem to play a role.

The spinal cord arises very early in fetal development–in the first several weeks of gestation. Many forms of spinal dysraphism develop during this time. Robust maternal nutrition early in pregnancy, especially adequate levels of a vitamin called folate, seems to protect against some forms of spinal dysraphism.

SPINAL CORD DEVELOPMENT-

Spinal cord development can be summarized in three basic embryologic stages – gastrulation (2–3 weeks), primary neurulation (3-4 weeks) and secondary neurulation (5–6 weeks). The rostral spinal cord (to about the level of S2) is formed by primary neurulation and the caudal spinal cord (distal to S2 level) by secondary neurulation, also referred to as canalization and retrogressive differentiation.

Gastrulation-

Gastrulation is the process of conversion of bilaminar disc into a trilaminar disc initiated by primitive streak. Primitive node, a depression at the cranial end of streak, contains cells that are important for organizing the embryonic axes. Epiblast cells migrate toward and through the streak and node, detach and form two new layers ventral to the remaining epiblast. The first cells through the streak displace the original hypoblast to form endoderm, whereas cells migrating slightly later create a new middle layer, the mesoderm. Nonmigrating cells of epiblast constitute the ectoderm. Some cells migrate cranially in the midline to form the prechordal plate and notochord, which initiate the process of neurulation by inducing the formation of the neural plate from overlying ectoderm cells. Thus, neural plate is derived from ectoderm and forms in the central part of this upper layer. Remainder of the ectoderm surrounding the neural plate forms the epidermis.

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PRIMARY NUTRITION-

Lateral borders of neural plate elevate into neural folds which later fuse in the midline to form the neural tube . The open regions of the neural tube called the anterior (cranial) and posterior (caudal) neuropores close by the zippering process. This neurulation process is called primary neurulation which is responsible for establishing brain and spinal cord regions down to the sacral levels (probably up to S2). On neural tube closure, overlying non-neural epidermal cells form the ectodermal layer of the skin. Normal neural tube closure occurs by day 25 to 27. Meanwhile, the neural tube separates from the overlying ectoderm, a process called dysjunction.

The neuroepithelial cells (neuroblasts) around the inner neural tube form the mantle layer, which produces the spinal cord gray matter. The outermost layer forms the marginal layer, which subsequently myelinates to produce the spinal cord white matter. The central neuroepithelial cells differentiate into ependymal cells along the central canal. Neural crest cells along each side of the neural tube form the dorsal root ganglia, autonomic ganglia, Schwann cells, leptomeninges and adrenal medulla.

SECONDARY NUTRITION-

The cord caudal to S2 level is formed by this process. Totipotent mesodermal cells called caudal cell mass or tail bud coalesce to form neural tube which then epithelialize, reorganize around a lumen and finally become continuous with the cranial part of the tube initially formed by primary neurulation. Part of the caudal cell mass undergoes both regression and differentiation (a process called retrogressive differentiation) to form filum terminale, terminal ventricle, tip of conus medullaris, and most of the sacrum, coccyx and coccygeal medullary vestige. By the third gestational month, spinal cord extends the entire length of the developing spinal column. Rapid elongation of the vertebral column and dura relative to the cord produces the apparent ascent of the cord during the remainder of the gestation and the conus is at the adult level soon after birth.

Clinico-radiologically, spinal dysraphism is classified into two categories. The first category is spinal dysraphism with back mass that is not covered by skin, i.e. open dysraphism. The second is spinal dysraphism with skin-covered back mass, i.e. closed dysraphism, which can be further subcategorized on the basis of the presence or absence of a subcutaneous mass.

Anomalies of Gastrulation-

Failure of notochord formation causes complex dysraphic states such as caudal regression syndrome and segmental spinal dysgenesis. Incorrect notochordal induction leads to the incomplete splitting of the neural plate from the notochord, producing the split notochord syndromes (neurenteric cyst and diastematomyelia).

Failure of notochord formation causes complex dysraphic states such as caudal regression syndrome and segmental spinal dysgenesis. Incorrect notochordal induction leads to the incomplete splitting of the neural plate from the notochord, producing the split notochord syndromes (neurenteric cyst and diastematomyelia).

Anomalies of notochord formation

Caudal regression syndrome

Caudal regression syndrome (CRS) is a complex dysraphic state with aberrations in gastrulation, secondary as well as primary neurulation. Most cases are sporadic, although a dominantly inherited defect in the HLXB9 gene has been described. Mothers of 15–20% of these infants are diabetic, and the offspring of 1% diabetic mothers are afflicted. Associations with other caudal spinal segment anomalies such as vertebral segmentation and formation anomalies and split cord malformations are noted.

Two types are described. Type 1 features a foreshortened terminal vertebral column, high-lying wedge-shaped conus termination and more severe associated visceral and orthopedic anomalies. Type 2 is less severe and has a low-lying tethered spinal cord with milder associated malformations In general, the higher the cord termination, the more severe is the sacral anomalies. The most severe CRS presentations are lumbosacral agenesis in which the spine terminates at the lower thoracic level and there is severe sacral dysgenesis with fused lower extremities in a “mermaid” configuration (sirenomyelia)

Segmental spinal dysgenesis (SSD) is a very rare dysraphic anomaly characterized by segmental thoracolumbar or lumbar vertebral and spinal cord dysgenesis or agenesis. Congenital thoracic or lumbar kyphosis is characteristic, with a palpable dorsal bone spur located at the gibbous apex. The upper spinal cord is normal, however, the cord segment below the dysgenetic segment is bulky, thickened and low-lying. The spinal canal proximal and distal to the dysgenetic level is of normal caliberSegmental spinal dysgenesis (SSD) is a very rare dysraphic anomaly characterized by segmental thoracolumbar or lumbar vertebral and spinal cord dysgenesis or agenesis. Congenital thoracic or lumbar kyphosis is characteristic, with a palpable dorsal bone spur located at the gibbous apex. The upper spinal cord is normal, however, the cord segment below the dysgenetic segment is bulky, thickened and low-lying. The spinal canal proximal and distal to the dysgenetic level is of normal caliberSegmental spinal dysgenesis (SSD) is a very rare dysraphic anomaly characterized by segmental thoracolumbar or lumbar vertebral and spinal cord dysgenesis or agenesis. Congenital thoracic or lumbar kyphosis is characteristic, with a palpable dorsal bone spur located at the gibbous apex.

Neurenteric cyst and dorsal-enteric spinal anomalies

Neurenteric cyst (NEC) is a complex dysraphic state and consists of an intraspinal cyst lined by enteric mucosa. It is most common in thoracic spine followed by cervical spine. They arise from an abnormal connection between primitive endoderm and ectoderm that persists beyond the third embryonic week. Normally, the notochord separates ventral endoderm (foregut) and dorsal ectoderm (skin, spinal cord) during embryogenesis, in an NEC, a separation failure “splits” the notochord and hinders the development of mesoderm, which traps a small piece of primitive gut within the developing spinal canal. This gut remnant may become isolated forming a cyst or it may maintain connections with gut or skin (or both); this produces the spectrum of fistulas and sinuses that constitute the spectrum of dorsal-enteric spinal anomalies.

The upper spinal cord is normal, however, the cord segment below the dysgenetic segment is bulky, thickened and low-lying. The spinal canal proximal and distal to the dysgenetic level is of normal caliber.

Abnormalities of Primary Neurulation

Premature dysjunction

If dysjunction occurs prematurely, perineural mesenchyme is interposed between neural tube and ectoderm, which may differentiate into fat and prevent complete neural tube closure. It leads to the lipomatous malformation spectrum of lipomyelocele, lipomyelomeningocele and spinal lipomas.

Lipomyelocele (LMC), b) lipomyelomeningocele (LMMC)

The main differentiating feature between a LMC and LMMC is the position of the placode–lipoma interface.With an LMC, the placode–lipoma interface lies within the spinal canal. With an LMMC, the placode–lipoma interface lies outside of the spinal canal due to expansion of the sub-arachnoid space and In both cases, syringomyelia is a commonly associated finding. LMC and LMMC account for 20–56% of occult spinal dysraphism and 20% of skin-covered lumbosacral masses. An important imaging point is that the neural placode is frequently rotated; this foreshortens the roots on one side, predisposing them to stretch injury, and lengthens the roots on the other side, rotating them into the surgeon’s field of view and making them more prone to injury.

The spinal lipoma

The spinal lipoma is a simple dysraphic state and is subdivided into intradural and terminal (filar) lipomas. An intradural lipoma refers to a lipoma located along the dorsal midline that is contained within the dural sac. No open spinal dysraphism is present. They are most commonly lumbosacral in location. Fibrolipomatous thickening of the filum terminale is referred to as a filar lipoma. Filar lipomas can be considered a normal variant if there is no clinical evidence of tethered-cord syndrom.

Nondysjunction

Nondysjunction results from failure of dissociation of neural tube from adjacent cutaneous tissue. If dysjunction fails to occur, an ectodermal–neuroectodermal tract forms that prevents mesenchymal migration. Nondysjunction results in open neural tube defect spectrum of dorsal dermal sinus, myelomeningocele, and myeloceles.

Dorsal dermal sinus

The simplest of these is the dorsal dermal sinus connecting skin dimple to the dural sac, conus, or central spinal cord canal. The most common dermal sinus tract (DST) location is in the lumbosacral spine, followed by the occiput. In all dermal sinus cases, there is some degree of focal dysraphism, which may be as subtle as a bifid spinous process. The true congenital dorsal DST usually has an atypical dimple at the ostium that is large (>5 mm), often asymmetric, and remote (>2.5 cm) from the anus A. These features help distinguish the dermal sinus from its clinically asymptomatic mimic, simple coccygeal dimple. The sinus tract/cord is epithelial-cell lined and may or may not be canalized. When patent, it exposes the patient to an elevated risk of meningitis. It is critical to look for this anomaly in all patients with atypical skin dimples, cutaneous back lesions or lipomas. Moreover, 30–50% of DSTs may have an associated dermoid or epidermoid cyst.

Myelomeningocele and myelocele

Myelomeningoceles and myeloceles are caused by defective closure of the primary neural tube and are clinically characterized by exposure of the neural placode through a midline skin defect on the back, and hence, classified under open dysraphic states. Myelomeningoceles account for more than 98% of open spinal dysraphisms. Myeloceles are rare. It is important to note that preoperative imaging of myelomeningocele is usually not done because of the risk of infection. Nevertheless, the main differentiating imaging feature between a myelomeningocele and myelocele is the position of the neural placode relative to the skin surface. The neural placode protrudes above the skin surface with a myelomeningocele and is flush with the skin surface with a myelocele. Myelomeningocele is almost always seen in the context of a Chiari 2 malformation.

Combined Anomalies of Gastrulation and Primary Neurulation

Hemimyelomeningocele and hemimyelocele

Hemimyelomeningoceles and hemimyeloceles can also occur but are extremely rare. These conditions occur when a myelomeningocele or myelocele is associated with diastematomyelia (cord splitting) and one hemicord fails to neurulate.

Anomalies of Secondary Neurulation/anomalies of the Caudal Cell Mass

Failure of expected secondary neurulation leads to conditions such as abnormally long spinal cord, tethered cord syndrome, persisting terminal ventricle, terminal myelocystocele, lipoma of filum terminale and intrasacral – anterior sacral meningocele. It is also implicated in pathogenesis of caudal regression syndrome and segmental spinal dysgenesis.

Persistent terminal ventricle/fifth ventricle

By day 48, a transient ventriculus terminalis appears in the future conus. According to Coleman et al., evidence of a fifth ventricle not accompanied by other pathologies is a frequent finding that does not have pathological significance during the first 5 years of life.

Key imaging features include location immediately above filum terminale and lack of contrast enhancement, which differentiates this entity from other cystic lesions of the conus medullaris.

Tethered cord syndrome

Tethered cord syndrome (TCS) patients most likely present during periods of rapid somatic growth. It manifests clinically as gait spasticity, low back and leg pain that is worse in the morning, lower extremity sensory abnormalities, and/or bladder difficulties. On imaging, TCS strictly refers to patients with a low-lying cord and thickened filum [>1.5 mm] .

Intrasacral – anterior sacral meningocele

The term “intrasacral meningocele” is used to denote a sac lined by arachnoid which lies within an enlarged sacral spinal canal and is attached to the caudal termination of the dural sac by a pedicle that usually permits cerebrospinal fluid (CSF) flow from the tip of the subarachnoid space into the meningocele. Consistent with the possible congenital origin, intrasacral meningocele may occur in association with other anomalies such as sacral vertebral anomalies, diastematomyelia or TCS

Anterior meningoceles are usually presacral in location. It has a large anterior meningocele outpouching that traverses an enlarged sacral foramen and produces a presacral cystic mass. Most ASMs are sporadic but a minority show an inherited predisposition within the Currarino triad or in syndromes that feature dural dysplasia, such as neurofibromatosis type 1 (NF1) and Marfan syndrome.

Terminal myelocystocele

Herniation of a large terminal syrinx (syringocele) into a posterior meningocele through a posterior spinal defect is referred to as a terminal myelocystocele. The terminal syrinx component communicates with the central canal, and the meningocele component communicates with the subarachnoid space. The terminal syrinx and meningocele components do not usually communicate with each other.

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INTRODUCTION-

This study explored premorbid, neurocognitive, behavioral, and familial factors in preschoolers, ages 3–6, who experienced a mild to moderate traumatic brain injury (TBI). Twenty-nine children with TBI, 33 children with mild to moderate injuries to other body regions, and 34 non-injured children participated in the study. Neuropsychological assessments and behavioral measures were administered at the time of hospitalization and 6 months later. In comparison to the non-injury children, preschool-aged children with TBI had higher rates of premorbid behavior difficulties, lower premorbid cognitive functioning, and poorer development of pre-academic skills. In addition, parents of children with TBI reported greater situational issues and life stressors than parents of children in the non-injured group. Some neurocognitive recovery was evident in the TBI group, but no differences were recognized in behavioral and family measures at the 6-month follow-up. This study emphasizes the relative effects of premorbid characteristics in later practice of preschool children who sustain TBI.

The purpose of this study was to compare child, hospital course, and discharge characteristics by admitting unit, injury type, head Abbreviated Injury Scale (AIS), and Glasgow Coma Scale (GCS), and test congruence of AIS and GCS categories. Chart data were collected from seven hospitals on 183 preschool children with head injury (90 admitted to PICU, 93 to general care unit). Injury events included falls (n = 89, 49%), hit by car (n = 35, 19%), motor vehicle crashes (n = 26, 14%), bicycle crashes (n = 12, 7%), and blunt traumas (n = 21, 11%). Most children (68%) had head injuries only, 20% had other fractures, 5% had organ damage, and 7% had all three. Injury severity was measured by head AIS and GCS scores. Treatments and procedures included tubes/lines, blood/blood products, and medications. Children with head injuries only had fewer hospital days, less severe head injuries, and near normal GCS scores. They were less likely to have tubes/lines and medications. Children were discharged with medications (61%) and medical equipment (14%). Five children were discharged to long-term care facilities, and five were discharged to rehabilitation facilities. Concordance of head AIS and GCS categories occurred for only 50 (28%) children. Although the GCS is the gold standard for identifying changes in neurological status, it was not as helpful in representing hospital care. Head AIS injury categories clustered children in more homogeneous groups and better represented hospital care. Head AIS categories are better indicators of injury severity and care provided than GCS. Head injury AIS score may be an important addition to GCS for guiding care.

Traumatic brain injury (TBI), sometimes referred to as a silent epidemic, effects thousands of people each year. Incidence of pediatric TBI is 220 per 100,000. Head trauma is placed as the major cause of death in children, with a mortality rate of 10 per 100,000 children (Annegers, 1983). This mortality rate alone, although concerning, does not fully describe the impact of TBI. Approximately 100,000–170,000 children are hospitalized each year with a TBI, and six times that visit the emergency room (Eiben et al., 1984; Kraus, Fife, Cox, Ramstein, & Conroy, 1986). In their 1974 study, Kalsbeck, McLaurin, Harris, & Miller (1980) reported that the direct and indirect costs of pediatric TBI were as much as 348 million dollars per year. These statistics indicate that TBI is a major public health problem, and a significant health-related financial burden (Gotschall, 1993).

While there is substantial literature on the effects of mild to moderate TBI on school-aged children, relatively little is known about mild to moderate TBI in preschool-aged children. Preschool TBI is a complex condition, which may have different outcomes depending upon the premorbid status of the child, the functional status of the family, and the severity of the injury. Research into the effects of TBI in preschoolers may allow us to understand this common childhood disorder better. To date, the limited literature on the preschool age group has focused primarily on the outcomes of severe TBI, so studies on mild and moderate TBI are greatly needed.

Research into preschool TBI is also important because it promises to further our understanding of the developmental variability of TBI throughout childhood, and perhaps expand our knowledge of the complex interplay between age of injury, severity of injury, and mechanism of injury in acquired childhood brain injury. Both animal and human studies of brain injury suggest that the age at which injury was sustained is a major contributor to eventual outcome. In general, evidence does not support the notion that the young brain is less vulnerable to insult or recovers better than the mature brain. We do not know, however, whether this is true of all ages, etiologies, or severity levels of traumatic injuries. In a review of research on age factors in TBI, Fletcher and Levin (1988) found evidence for greater compromise in children less than 6 years of age. However, the worse outcome in the younger sample may have been colored by a high rate of TBI from abuse. It is now known that abused children may suffer more severe injuries, they may differ from non-abused children prior to the injury, and they tend to have a worse general outcome from traumatic brain injury due to pathophysiological factors unique to abuse (Goldstein, Kelly, Bruton, & Cox, 1993; Michaud, Rivara, Grady, & Reay, 1992; Trickett & Aber, 1991). When a sub-sample of physically abused children was removed from analyses, younger and older children with severe injury had similar outcomes (Kriel, Krach, & Panser, 1989). There are other studies not including abuse as a confound, which suggest somewhat worse outcome in younger children equated for severity of injury with older children (Ewing-Cobbs, Levin, Eisenberg, & Fletcher, 1987; Gulbrandsen, 1984; Levin, Eisenberg, Wigg, & Kobayashi, 1982), especially for preschoolers (Michaud, Rivara, Jaffe, Fay, & Dailey, 1993; Wrightson, McGinn, & Gronwall, 1995).

Wrightson et al. (1995) concluded that preschoolers with severe traumatic brain injury might fare far worse than older children. This may, in part, be due to the latency in symptom manifestation with the deficit taking years to become evident. These late cognitive effects are known as patients “growing into their deficits” (Grattan & Eslinger, 1991; Kolb, 1995). In a retrospective analysis of school-aged children, Michaud et al. (1993) reported that children in special education classes had a higher incidence of preschool head injury than children in regular classes. Similarly, Gronwall, Wrightson, and McGinn (1997) found that significantly more children who had a history of TBI in the preschool years needed special assistance with reading than children who had no injury prior to beginning school. The first study certainly suggests that younger children with severe injury fare more poorly than older children. The latter two suggest that preschoolers with milder injury fare more poorly as well, but these studies are retrospective in nature, inferring TBI sequelae years after the event. One case study suggested that preschoolers might be vulnerable to mild head injury and show delayed effects. PET and neuropsychological testing documented hypometabolism in both temporal lobes, and verbal and visual memory deficits, respectively, in a young child who sustained whiplash injury in a motor vehicle accident. Staring spells, brief confusional episodes, and emotional outbursts were noted 2 years after injury, and epileptiform activity was documented on EEG 4 years post-trauma (Roberts, Manshadi, Bushnell, & Hines, 1995).

The age at which the child is injured may also affect the manifestation of cognitive dysfunction. There is increasing evidence that skills in a rapid state of development at injury may be more vulnerable to the effects of severe TBI. Preschoolers are likely to demonstrate compromise in motor and expressive language skills (Ewing-Cobbs, Miner, Fletcher, & Levin, 1989) and school-aged children may be more compromised in reading (Shaffer, Chadwick, & Rutter, 1975) and written language (Ewing-Cobbs et al., 1987). The results of one retrospective study suggested that preschoolers had greater impairment in reading (Gronwall et al., 1997).

A prospective study of preschoolers with mild to moderate TBI can help assess whether recovery patterns parallel that of older children and adults, as reported in the literature. Initial cognitive deficit is common in older children, but there is little evidence of sustained neurocognitive or learning deficits in the majority of children (Asarnow et al., 1995; Bawden, Knights, & Winogren, 1985; Bijur & Haslum, 1995; Chadwick, Rutter, Brown, Shaffer, & Traub, 1981a; Gulbrandsen, 1984, Levin et al., 1988). Again, some of the research suggests that preschoolers may be especially vulnerable even to mild TBI.

Rutter, Chadwick, Shaffer, & Brown (1980) have further suggested that many of the deficits evident in children with a history of TBI predated the injury. Studies have documented pre-existing developmental difficulties and specific learning problems (Klonoff & Paris, 1974), language problems (Mahoney et al., 1983), and lower academic achievement (Chadwick, Rutter, Brown, Shaffer, & Taub, 1981b) in preschoolers who sustain TBI. Demographic risk factors for all childhood injury include poverty, single-family households, and congested living conditions. Psychiatric histories, drug/alcohol histories, and physical illness are found more frequently in the parents of injured children. Unsupervised play is also reported more frequently, which may be directly related to incidence of injury (Chadwick et al., 1981b; Klonoff & Paris, 1974). It is possible that pre-existing cognitive problems will also affect incidence of injury due to the child’s inability to evaluate risks and dangers.

Many studies also suggest the child with traumatic brain injury is more likely to have a history of behavioral disorder (Bijur & Haslum, 1995; Brown, Chadwick, Shaffer, Rutter, & Traub, 1981; Chadwick et al., 1981b; Klonoff, 1971; Klonoff & Paris, 1974). In a sample of 100 pediatric patients suffering traumatic brain injury, Arffa (1995) found that 55% had a history of either school learning difficulties, behavioral problems, or emotional disturbance. Child behavioral characteristics associated with high traumatic brain injury rates include impulsivity, aggression, and attention seeking behavior. These characteristics might also be seen as proximal causes of injury. Behavioral factors in the toddler are secondary to home environment in the injury equation (Matheny, 1987). A previous history of traumatic brain injury is also associated with an increased risk of further traumatic brain injury (Annegers, 1983). Multiple TBI’s are strongly related to socioeconomic factors and behavioral characteristics such as hyperactivity and aggression (Bijur & Haslum, 1995).

Some studies have found an interaction between new behavioral disturbance and TBI severity, but variation among studies occur depending on the criteria used to describe behavior problems or change (Brink, Imbus, & Woo-Sam, 1980; Fletcher, Ewing-Cobbs, Miner, Levin, & Eisenberg, 1990; Rivara et al., 1994). In the studies by Rutter and colleagues, the rate of new psychiatric disorder was three times more common in severely head injured children than in orthopedic injury controls (Brown et al., 1981; Rutter, Chadwick, & Shaffer, 1983). Behavioral disposition may actually worsen over time in severe injury (Fletcher, Ewing-Cobbs, Francis, & Levin, 1995). A severity threshold was proposed as psychiatric disorder became much more likely in very severe injury (post-traumatic amnesia for more than 22 days) than in severe (7–21 days of post-traumatic amnesia) or mild injuries. Threshold effects noted in other studies (Levin & Eisenberg, 1979) were lower than that of Brown and Rutter. No significant difference in rate of new psychiatric disorder was observed in children with mild injuries when contrasted with orthopedic control groups (Brown et al., 1981, Fletcher et al., 1990, Rutter et al., 1983). Pre-injury behavior was found to be related to behavioral outcome in Rutter’s studies. Over one-half with a doubtful disorder developed a definable disorder and all had symptoms. A study by Rivara et al. (1993) yielded similar findings, reporting child behavior ratings obtained 1 year after TBI correlated with pre-injury ratings of child behavior.

Family functioning may be an important mediator of behavioral outcome in children with TBI, perhaps more important than neurocognitive function. Brown et al. (1981) found that children from problematic family backgrounds also were at high risk for developing new psychiatric disorders following the injury. Rivara et al. (1992) found that family functioning interacted with premorbid child characteristics. A deterioration of family functioning for children with severe TBI relative to children with mild or moderate TBI was also evident. Taylor et al. (1995) emphasized a link between child behavior problems and family stress, negative life events, and parental psychological symptoms. Perrott, Taylor, & Mantes (1991) proposed that family dysfunction may have adverse effects on subsequent child behavior and adaptation in spite of continuing cognitive recovery.

The present study was designed as a comprehensive exploration of the premorbid states of preschool-aged children with TBI, the immediate cognitive effects of TBI, and the post-acute cognitive and behavioral outcomes of TBI.

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Materials and Methods-

Setting and Procedure

Hospital records of 183 preschool children (ages 36 through 83 months) who suffered a head injury were reviewed after parental consent. Children were hospitalized in one of seven tertiary care centers, including three free-standing children’s hospitals. Admission to the pediatric intensive care unit (PICU) versus a general care unit (GCU) was decided by the admitting physicians (not involved in the study) and hospital policy. Institutional Review Board approvals were obtained from the appropriate universities and hospitals.

For this research, head injury was defined as an injury event where a blow to the head was probable with at least one physical finding suggesting head trauma, including symptoms of head injury in children: loss of consciousness no matter how brief, emesis, drowsiness, seizures, neurological deficits, cerebrospinal fluid or bloody discharge from the ears or nose, or a positive CT scan or skull X-ray. To be eligible, the injured child was a) free from chronic illnesses other than asthma, b) not previously hospitalized other than at birth, and c) without severe pre-existing cognitive deficits. For purposes of the larger study, children had to be living with at least one biological or adoptive parent before the injury and expected to survive. Parents had to understand spoken English. Exclusion criteria necessary for the larger study were a) injury suspected to be due to child abuse, b) child meeting or being evaluated with brain death criteria, c) parent(s) hospitalized concurrently with major injury, or d) death of parent(s) in the injury event.

For this research, head injury was defined as an injury event where a blow to the head was probable with at least one physical finding suggesting head trauma, including symptoms of head injury in children: loss of consciousness no matter how brief, emesis, drowsiness, seizures, neurological deficits, cerebrospinal fluid or bloody discharge from the ears or nose, or a positive CT scan or skull X-ray. To be eligible, the injured child was a) free from chronic illnesses other than asthma, b) not previously hospitalized other than at birth, and c) without severe pre-existing cognitive deficits. For purposes of the larger study, children had to be living with at least one biological or adoptive parent before the injury and expected to survive. Parents had to understand spoken English. Exclusion criteria necessary for the larger study were a) injury suspected to be due to child abuse, b) child meeting or being evaluated with brain death criteria, c) parent(s) hospitalized concurrently with major injury, or d) death of parent(s) in the injury event.

Instruments

Injury severity was measured through hospital record review with the Abbreviated Injury Scale (AIS) and the Glasgow Coma Scale (GCS). Severity of the anatomical injuries for the AIS was determined with reports of clinically indicated procedures (X-ray, CT, magnetic resonance imaging [MRI], laboratory, operative) and chart notes recorded at the time of care by emergency room staff, emergency medical service providers (when available), and admitting physicians and nurses. Most GCS scores were recorded in the chart as part of the clinical care.

The AIS classifies severity of individual anatomical injuries by body regions, and therefore, does not change over time. Severity of injury in six body regions (head/neck, face, chest, abdomen, extremities/pelvic girdle, and external) is scored from one to six. The Injury Severity Scale (ISS) total score is calculated by summing the squares of the highest AIS code in the three body regions with the most severe injury. Severity scores for the AIS for a specific body region and the total ISS score are categorized as minor/mild, moderate, serious, severe, critical, and major/grave (AAAM, 1990). Children with “major/grave” injuries rarely survive, making them ineligible for the larger study. The severe and critical categories were combined because of the small number in each category. For this analysis, children’s head AIS scores were used to characterize the severity of head injury from mild to severe/critical. Three children’s head injuries were not scored because of lack of sufficient specific information in the chart to make a determination.

Results

2.1. Behavioral measures: premorbid estimates

2.1.1. Parenting Stress Index (PSI)

An analysis of variance (ANOVA) showed a significant difference between groups on the Life Stress Scale (F(2,86) = 4.2, p < .05), and Tukey’s post hoc analysis indicated that parents of TBI children reported significantly more stress than the children in the OI group (p < .05) and the NC group (p < .001). An ANOVA showed a significant difference between groups on the Defensive Responding Scale (F(2,86) = 4.2, p < .05), with the parents in the TBI group having a significantly higher rate of defensive responding than the parents in the NC group, suggesting that parents of TBI children may have underreported symptoms. Since analysis of the Defensive Responding Scale of the PSI indicated the possibility of parental underreporting of symptoms, correlations between scores on the Defensive Responding Scale and scores on all parent report questionnaires were examined. Significant correlations were followed by analyses of covariance to adjust statistically for between-group differences in defensive responding.

A multivariate analysis of covariance (MANCOVA), using the Defensive Responding Scale as a covariate, identified a significant covariate and group effect for the overall Child Domain Scale (F = (2,87) = 25.4, p < .001, r = .69; F = (2,86) = 7.8, p < .001) and Total Stress Scale (F = (2,87) = 53.8, p < .001, r = .63; F = (2,86) = 5.9, p < .001), but not the overall Parent Domain Scale. In addition, there were significant covariate and group effects for the Child Domain subtests of distractibility/hyperactivity (F = (2,87) = 16.7, p < .001, r = .44; F = (2,86) = 5.1, p < .005), adaptability (F = (2,87) = 18.7, p < .001, r = .48; F = (2,86) = 6.6, p < .005), demandingness (F = (2,87) = 21.1, p < .001, r = .51; F = (2,86) = 9.2, p < .001), and acceptibility (F = (2,87) = 22.8, p < .001, r = .48; F = (2,86) = 3.4, p < .05), as well as on the Parent domain subtests of Competence (F = (2,87) = 25.3, p < .001, r = .53; F = (2,86) = 8.3, p < .001) and Health (F = (2,87) = 37.6, p < .001, r = .59; F = (2,86) = 3.9, p < .05). Bonferroni post hoc contrasts revealed that children in the TBI group scored significantly higher than children in the NC group on distractibility/hyperactivity, adaptability, and acceptability. Children in the TBI group and OI group were found to be more demanding than children in the NC group. Parents from the NC group appeared to feel more confident in their parenting than parents in the TBI group and OI group. Parents from the OI group reported significantly better health than parents from the TBI group. displays the means, standard deviations, and p-values.

Table 2. MANCOVA group mean summary table on the PSI: acute testing

PSI scales	Group	p
	TBI	Other injury	Non-injury
	M	M	M
Total stress	35.44	38.61	30.65	.004**
Child domain	50.08	45.14	33.44	.001**
Parent domain	29.28	33.43	33.62	.262
Child domain subscales
Adaptability	57.48	44.18	39.88	.002**
Acceptability	47.36	44.25	39.26	.038*
Demandingness	54.04	51.54	35.35	.000***
Mood	59.48	53.61	53.50	.183
Distractibility/hyperactivity	47.00	40.43	32.03	.008**
Reinforces parent	52.12	49.36	50.65	.466
Parent domain subscales
Depression	28.56	36.07	34.50	.215
Attachment	44.04	40.39	35.97	.219
Role restriction	28.68	32.25	45.50	.635
Competence	33.72	35.11	22.12	.001**
Isolation	41.92	43.50	52.91	.644
Spouse	43.28	43.07	46.76	.468
Health	49.32	36.14	50.56	.024*
Defensive responding	0.44	0.32	0.12	.018*
Life stress	67.48	43.59	27.26	.000***

Note: means were adjusted using defensive responding as a covariate. ^*p < .05, ^**p < .01, ^***p < .001

The importance of early childhood experiences for brain development

Children are born ready to learn, and have many skills to learn over many years. They depend on parents, family members, and other caregivers as their first teachers to develop the right skills to become independent and lead healthy and successful lives. How the brain grows is strongly affected by the child’s experiences with other people and the world. Nurturing care for the mind is critical for brain growth. Children grow and learn best in a safe environment where they are protected from neglect and from extreme or chronic stressexternal icon with plenty of opportunities to play and explore.

Parents and other caregivers can support healthy brain growth by speaking to, playing with, and caring for their child. Children learn best when parents take turns when talking and playing, and build on their child’s skills and interests. Nurturing a child by understanding their needs and responding sensitively helps to protect children’s brains from stress. Speaking with children and exposing them to books, stories, and songs helps strengthen children’s language and communication, which puts them on a path towards learning and succeeding in school.

Exposure to stress and trauma can have long-term negative consequences for the child’s brain, whereas talking, reading, and playing can stimulate brain growth. Ensuring that parents, caregivers, and early childhood care providers have the resources and skills to provide safe, stable, nurturing, and stimulating care is an important public health goal.

When children are at risk, tracking children’s development and making sure they reach developmental milestones can help ensure that any problems are detected early and children can receive the intervention they may need.

Learn more about supporting early childhood experiences:

• Tracking developmental milestones
• Preventing abuse and neglect
• Positive parenting tips
• Healthy childcareexternal icon

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A healthy start for the brain

To learn and grow appropriately, a baby’s brain has to be healthy and protected from diseases and other risks. Promoting the development of a healthy brain can start even before pregnancy. For example, a healthy diet and the right nutrients like sufficient folic acid will promote a healthy pregnancy and a healthy nervous system in the growing baby. Vaccinations can protect pregnant women from infections that can harm the brain of the unborn baby.

During pregnancy, the brain can be affected by many types of risks, such as by infectious diseases like Cytomegalovirus  or Zika virus, by exposure to toxins, including from smoking or alcohol, or when pregnant mothers experience stress, trauma, or mental health conditions like depression. Regular health care during pregnancy can help prevent complications, including premature birth, which can affect the baby’s brain. Newborn screening can detect conditions that are potentially dangerous to the child’s brain, like phenylketonuria (PKU). external icon

Healthy brain growth in infancy continues to depend on the right care and nutrition. Because children’s brains are still growing, they are especially vulnerable to traumatic head injuries, infections, or toxins, such as lead. Childhood vaccines, such as the measles vaccine, can protect children from dangerous complications like swelling of the brain. Ensuring that parents and caregivers have access to healthy foods and places to live and play that are healthy and safe for their child can help them provide more nurturing care.

Learn more about the recommended care:

• Before pregnancy
• During pregnancy
• Around birth
• During infancy
• During early childhood

Initiation of physical, occupational, and speech therapy in children with traumatic brain injury

Abstract

Objectives: (1) To determine factors associated with physical therapy (PT) or occupational therapy (OT) evaluation and speech or swallow therapy evaluation in hospitalized children with traumatic brain injury (TBI); (2) to describe when during the hospital stay the initial therapy evaluations typically occur; and (3) to quantify any between-hospital variation in therapy evaluation.

Design: Retrospective cohort study.

Setting: Children’s hospitals participating in the Pediatric Health Information System database (January 2001-June 2011).

Participants: Children (age <18y) with TBI (N=21,399) who were admitted to the intensive care unit and survived to hospital discharge.

Interventions: Not applicable.

Main outcome measures: PT or OT evaluation and speech or swallow therapy evaluation. A propensity score was calculated to allow comparison of expected with observed rates of therapy evaluations by the hospital.

Results: The median hospital length of stay was 5 days (interquartile range, 3-10d). Overall, 8748 (41%) of 21,399 children received either a PT or OT evaluation, and 5490 (26%) out of 21,399 children received either a speech or swallow evaluation. Older children and those with higher energy injury mechanisms, more severe injuries, extremity fractures, more treatment with neuromuscular blocking agents or pentobarbital, and admission to a hospital with an American College of Surgeons Level I pediatric trauma designation were more likely to receive therapy evaluations. The median time until the first therapy evaluation was 5 (PT or OT) and 7 days (speech or swallow). Expected hospital evaluation rates were 25% to 54% (PT or OT) and 16% to 35% (speech or swallow), while observed hospital evaluation rates were 11% to 74% (PT or OT) and 4% to 55% (speech or swallow).

Conclusions: There is wide between-hospital variation in provision of rehabilitation therapies for children with TBI. Evidence-based criteria for initiation of routine therapy evaluations after TBI are needed.

Abstract

Objective: To describe the use of occupational therapy (OT), physical therapy (PT), and speech therapy (ST) treatment activities throughout the acute rehabilitation stay of patients with traumatic brain injury.

Design: Multisite prospective observational cohort study.

Setting: Inpatient rehabilitation settings.

Participants: Patients (N=2130) admitted for initial acute rehabilitation after traumatic brain injury. Patients were categorized on the basis of admission FIM cognitive scores, resulting in 5 fairly homogeneous cognitive groups.

Interventions: Not applicable.

Main outcome measures: Percentage of patients engaged in specific activities and mean time patients engaged in these activities for each 10-hour block of time for OT, PT, and ST combined.

Results: Therapy activities in OT, PT, and ST across all 5 cognitive groups had a primary focus on basic activities. Although advanced activities occurred in each discipline and within each cognitive group, these advanced activities occurred with fewer patients and usually only toward the end of the rehabilitation stay.

Conclusions: The pattern of activities engaged in was both similar to and different from patterns seen in previous practice-based evidence studies with different rehabilitation diagnostic groups.

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