Amyotrophic lateral sclerosis

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By Jinsy A Andrews MD and Hiroshi Mitsumoto MD

Amyotrophic lateral sclerosis (ALS) is also known as or subsumes Lou Gehrig disease, Primary lateral sclerosis, Aran-Duchenne disease, Familial amyotrophic lateral sclerosis, Flaccid bulbar palsy, Progressive bulbar palsy, Progressive crural palsy, Progressive muscular atrophy, and Spastic bulbar palsy -ed.

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease characterized by progressive muscle weakness without notable sensory loss. Although studies show that riluzole prolongs life only 2 to 3 months, it is the only U.S. FDA-approved medicine for this condition. In this clinical summary, Dr. Jinsy A Andrews, Director of Research and the Clinical Trials Unit at Hospital for Special Care, and Dr. Hiroshi Mitsumoto, Director of the Eleanor and Lou Gehrig MDA/ALS Center at Columbia University Medical Center, review clinical manifestations, provide updates on recent genetic discoveries, discuss recent reports of head trauma and motor neuron disease, discuss recent advances in technology, including the FDA approval of diaphragm pacing for ALS and brain computer interfaces (BCI), and cover the recent initiation of the ALS registry.

In This Article

Historical note and nomenclature
Clinical manifestations
Clinical vignette
Pathogenesis and pathophysiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
References cited