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Amyotrophic lateral sclerosis (ALS) is a heterogenous neurodegenerative disease characterized clinically by progressive muscle weakness and wasting caused by upper and lower motor neuron death usually ending in ventilatory failure.1 The cause of sporadic ALS, the most common form of ALS, is unknown, although genetic and environmental factors are implicated in increasing susceptibility of motor neurons to protein aggregation and RNA malfunction. Resultant cellular stress and an interplay of inflammatory and excitotoxic changes culminate in neuronal cell death.2
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See page 1042
- © 2019 American Academy of Neurology
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