ABSTRACT
Amyotrophic lateral sclerosis (ALS) remains a fatal disorder characterized by progressive paralysis terminating in an inevitable loss of respiratory function and death within five years of diagnosis. The most common motor neuron disease in adults, ALS-known through history as Charcot’s sclerosis and Lou Gehrig’s disease-has as its signature neuropathologic effect the selective and progressive loss of motor neurons in the brain, brainstem, and spinal cord (1). The clinical hallmarks are muscle atrophy, weakness, and fasciculations-or lower motor neuron syndrome-along with attendant long tract signs of hyperactive tendon reflexes, clonus, and Hoffman’s and Babinski’s signs reflecting the clinical manifestations of the lateral sclerosis observed at autopsy. Disease specificity is highlighted by the complete absence of cognitive impairment in the disease despite the near-complete paralysis of extremities and muscles of speech and eventually of respiration.
