What Is Motor Neuron Disease?

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By jolene
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Reviewed: Dr. Gromatzky
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5. Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is also known as Lou Gehrig disease. It is a disease that causes the death of neurons that control voluntary muscles. It is the most common neurodegenerative disease of motor neuron disease in adults.

Characterized by muscle wasting or atrophy, fasciculations, and spasticity, there is progressive skeletal muscle weakness and results in fatality. The symptoms occur as a result of degeneration of both upper and lower motor neurons leading to respiratory paralysis and death. There are three types of ALS: familial ALS, sporadic ALS, and Western Pacific ALS. Most cases of ALS are sporadic with 5% to 10% considered to be familial. In 30% to 40% of familial ALS cases, mutations in the C9orf72 gene has been reported.

Motor Neuron Disease

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