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Sci. Aging Knowl. Environ., 3 September 2003
Vol. 2003, Issue 35, p. dn2
[DOI: 10.1126/sageke.2003.35.dn2]


Amyotrophic Lateral Sclerosis

Paul H. Gordon, Hiroshi Mitsumoto, and Arthur P. Hays

The authors are at the Eleanor and Lou Gehrig Muscular Dystrophy Association/Amyotrophic Lateral Sclerosis Research Center, Department of Neurology (P.H.G. and H.M.), and Department of Pathology (A.P.H.), College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA. E-mail: PGordon{at} (P.H.G.);2003/35/dn2

Key Words: amyotrophic lateral sclerosis • motor neuron disease • neurodegeneration

Abstract: In this case study, we describe the symptoms, neurological examination, and pathology of a woman with amyotrophic lateral sclerosis (ALS). ALS is a rare disorder leading to degeneration of the voluntary motor system and death in, on average, 3 to 4 years. The loss of motor neurons in the brain and spinal cord causes the progressive symptoms of muscular weakness, atrophy, fasciculation (muscle twitching), spasticity, and hyperreflexia. Signs of disease in both upper and lower motor neurons are required for a definitive clinical diagnosis. Pathology shows degeneration of the lateral corticospinal tracts, loss of motor neurons and astrogliosis in the brain and brain stem, and neuronal inclusions. This case was marked by the onset of weakness and muscle atrophy in the hand, which spread to involve contiguous muscle segments. Cognition, the extraocular muscles, and the urinary sphincters were spared. Respiratory muscle weakness was a late manifestation.

Citation: P. H. Gordon, H. Mitsumoto, A. P. Hays, Amyotrophic Lateral Sclerosis. Sci. SAGE KE 2003 (35), dn2 (2003).

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