{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T10:50:28Z","timestamp":1773053428759,"version":"3.50.1"},"reference-count":7,"publisher":"American Association for the Advancement of Science (AAAS)","issue":"35","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Sci. Aging Knowl. Environ."],"published-print":{"date-parts":[[2003,9,3]]},"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.1126\/sageke.2003.35.dn2","type":"journal-article","created":{"date-parts":[[2003,9,23]],"date-time":"2003-09-23T07:50:31Z","timestamp":1064303431000},"source":"Crossref","is-referenced-by-count":7,"title":["Amyotrophic Lateral Sclerosis"],"prefix":"10.1126","volume":"2003","author":[{"given":"Paul H.","family":"Gordon","sequence":"first","affiliation":[{"name":"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.(P.H.G.)"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroshi","family":"Mitsumoto","sequence":"additional","affiliation":[{"name":"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.(P.H.G.)"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arthur P.","family":"Hays","sequence":"additional","affiliation":[{"name":"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.(P.H.G.)"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"221","reference":[{"key":"e_1_3_1_1_1","doi-asserted-by":"crossref","unstructured":"J. Andersen Defects in dynein linked to motor neuron degeneration in mice. Sci. SAGE KE 2003 pe10 (2003). [Abstract] [Full Text]","DOI":"10.1126\/sageke.2003.18.pe10"},{"key":"e_1_3_1_2_1","unstructured":"M. Mitsumoto D. A. Chad E. P. Pioro Amyotrophic Lateral Sclerosis (Davis Philadelphia PA 1998)."},{"key":"e_1_3_1_3_1","doi-asserted-by":"crossref","unstructured":"H. Mitsumoto Clinical trials: present and future. Amyotroph. Lateral Scler. Other Motor Neuron Disord. 2 (suppl. 1) S10-S14 (2001). [Abstract]","DOI":"10.1080\/14660820152415672"},{"key":"e_1_3_1_4_1","doi-asserted-by":"crossref","unstructured":"T. L. Munsat P. L. Andres L. Finison T. Conlon L. Thibodeau The natural history of motor neuron loss in amyotrophic lateral sclerosis. Neurology 38 409-413 (1988). [Abstract]","DOI":"10.1212\/WNL.38.3.409"},{"key":"e_1_3_1_5_1","doi-asserted-by":"crossref","unstructured":"S. Przedborski H. Mitsumoto L. P. Rowland Recent advances in amyotrophic lateral sclerosis research. Curr. Neurol. 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[Abstract]","DOI":"10.1056\/NEJM200105313442207"}],"container-title":["Science of Aging Knowledge Environment"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.science.org\/doi\/full\/10.1126\/sageke.2003.35.dn2","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,9]],"date-time":"2024-01-09T19:28:49Z","timestamp":1704828529000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.science.org\/doi\/10.1126\/sageke.2003.35.dn2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2003,9,3]]},"references-count":7,"journal-issue":{"issue":"35","published-print":{"date-parts":[[2003,9,3]]}},"alternative-id":["10.1126\/sageke.2003.35.dn2"],"URL":"https:\/\/doi.org\/10.1126\/sageke.2003.35.dn2","relation":{},"ISSN":["1539-6150","1539-6150"],"issn-type":[{"value":"1539-6150","type":"print"},{"value":"1539-6150","type":"electronic"}],"subject":[],"published":{"date-parts":[[2003,9,3]]}}}