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Amyotrophic Lateral Sclerosis (ALS)


Definition

  • Progressive neuromuscular disease characterized by degeneration of upper and lower motor neurons resulting in progressive skeletal muscle wasting and weakness leading to respiratory failure and death; ALS subtypes: primary lateral sclerosis (mainly upper motor disease) and progressive muscular atrophy (mainly lower motor disease); synonymous with motor neuron disease

Clinical Features

Epidemiology

  • Incidence: 2 per 100,000 individuals/year; prevalence of about 5 per 100,000 individuals

  • Mean age of onset for sporadic ALS (90% of cases) is 60 years; 25% of cases present before age 50

  • Familial ALS in about 10%

  • Sporadic disease more common in males (male-to-female ratio is 1.5 : 1), but no gender preference in familial disease

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