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Motor neurones are cells in the brain and spinal cord that allow us to move, speak, swallow and breathe by sending commands from the brain to the muscles that carry out these functions. Their nerve fibers are the longest in the body, a single axon can stretch from the base of the spinal cord all the way to the toes.
Motor neurons divided into either upper or lower motor neurones, forming various tightly controlled, complex circuits throughout the body. This controls both voluntary and involuntary movements through the innervation of effector muscles and glands. The upper and lower motor neurons form a two-neuron circuit.
- The upper motor neurons originate in the cerebral cortex and travel down to the brain stem or spinal cord
- The lower motor neurons begin in the spinal cord and go on to innervate muscles and glands throughout the body.
Upper and lower motor neurons utilize different neurotransmitters to relay their signals.
- Upper motor neurons use glutamate
- Lower motor neurons use acetylcholine.
Causes
Conditions that damage upper motor neurons include:
- Lou Gehrig’s disease (amyotrophic lateral sclerosis, or ALS)
- Primary lateral sclerosis (PLS)
- Traumatic brain injury
- Spinal cord injury
- Multiple sclerosis
- Stroke
- Huntington’s disease

Symptoms
Upper motor neuron lesions prevent signals from traveling from your brain and spinal cord to your muscles. Your muscles can’t move without these signals and become stiff and weak.
Damage to upper motor neurons leads to a group of symptoms called upper motor neuron syndrome:
Muscle weakness. The weakness can range from mild to severe.
Overactive reflexes. Your muscles tense when they shouldn’t. For example, just rubbing your hand over your belly might cause your abdominal muscles to tighten up.
Tight muscles. The muscles become rigid and hard to move.
Clonus. This is muscular spasm that involve repeated, often rhythmic, contractions.
The Babinski response. Young children have a reflex called the Babinski reflex. If you stroke the bottom of their foot, their big toe will pull back and their other toes will spread out. This reflex usually disappears after age 2. In adults, the Babinski response is a sign of damage to the nervous system.
Upper motor neuron lesions can get worse over time. Over time, you can have trouble controlling your muscles.
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Diagnosis
Motor neuron diseases can be tricky to diagnose. Their symptoms are often very similar to those of other diseases.
Your doctor can do blood and urine tests to check for infections, muscle diseases, and other conditions that have symptoms similar to those of motor neuron diseases.
During an exam, your doctor will look for signs of a nervous system problem by checking your:
- Balance and coordination
- Movement
- Hearing, speech, and vision
- Memory and concentration
A few other tests can help your doctor diagnose upper motor neuron lesions:
MRI, or magnetic resonance imaging. It uses powerful magnets and radio waves to make pictures of structures inside your body. An MRI can show damage to upper motor neurons.
EMG, or electromyogram. It uses a thin needle to check the activity in your muscles when they contract and when they’re at rest. An EMG can check for problems with your lower motor neurons and help diagnose ALS and PLS.
Nerve conduction study. This test measures how quickly an electrical current moves through your nerve. It can show how well your nerves are sending signals to your muscles and if you have nerve damage.
Spinal tap or lumbar puncture. It removes a small amount of fluid from your spine to show whether MS or an infection is causing your symptoms.
Nerve biopsy. It removes a small sample of the nerve to check for damage. It’s not likely that you’ll have this done. Doctors hardly ever use this method when trying to diagnose an upper motor neuron disease.
Treatment
Which treatment you get depends on what disease caused your upper motor neuron lesions.
Medicines won’t stop diseases like ALS or PLS, but they can help you manage symptoms. Some of the drugs used to treat upper motor neuron symptoms include:
Muscle relaxants.Baclofen, clonazepam (Klonopin), and tizanidine (Zanaflex) control muscle spasms in PLS. Doctors may also use Botox to treat tightness and stiffness of muscles.
ALS drugs. Edaravone (Radicava) and riluzole (Rilutek) slow the progression of ALS.
MS drugs. drugs which can slow MS damage to nerve cells. Beta interferons, alemtuzumab (Lemtrada), cladribine (Mavenclad), dimethyl fumarate (Tecfidera), diroximel fumarate (Vumerity), fingolimod (Gilenya), glatiramer acetate (Copaxone), mitoxantrone (Novantrone), monomethyl fumarate (Bafiertam), natalizumab (Tysabri), ocrelizumab (Ocrevus), ozanimod (Zeposia), siponimod (Mayzent), teriflunomide (Aubagio)
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