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Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS), also known as motor neurone disease (MND) or Lou Gehrig's disease, is a neurodegenerative disease that results in the progressive loss of motor neurons that control voluntary muscles.

Amyotrophic lateral sclerosis can affect nerve cells in the brain and spinal cord, causing a loss of muscle control. 2% of neurofilament accumulation genetics cases have SOD1 mutations that probably act through a toxic property.

Incidence (average 1.89 per 100,000/year) and prevalence (average 5.2 per 100,000) are relatively uniform in Western countries, although foci of higher frequency occur in the Western Pacific. The mean age of onset for sporadic amyotrophic lateral sclerosis is about 60 years. Approximately two-thirds of patients with typical amyotrophic lateral sclerosis have a spinal form of the disease (limb onset) and present with symptoms related to focal muscle weakness and wasting, where the symptoms may start either distally or proximally in the upper and lower limbs. Gradually, spasticity may develop in the weakened atrophic limbs, affecting manual dexterity and gait.


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