Document Detail

Sporadic amyotrophic lateral sclerosis of long duration mimicking spinal progressive muscular atrophy: a clinicopathological study.
MedLine Citation:
PMID:  10202983     Owner:  NLM     Status:  MEDLINE    
We report an autopsy case of amyotrophic lateral sclerosis (ALS) clinically diagnosed as spinal progressive muscular atrophy (SPMA). The patient was a Japanese woman without hereditary burden. She developed muscle weakness of the distal part of the left lower extremity at age 42, followed by muscle weakness and atrophy of the right lower extremity and upper extremities. At age 57, she needed transient ventilatory support. Slight weakness in the facial muscles and fasciculation of the tongue appeared at age 60. At age 61, she died of sudden respiratory arrest. During the clinical course, neurological examination revealed neither Babinski signs nor hyperreflexia. The neuropathological examination revealed not only neuronal loss with gliosis in the facial nucleus, hypoglossal nucleus, and anterior horns of the spinal cord, but also loss of Betz cells and degeneration of the pyramidal tracts. Based on these clinicopathological findings and review of literature, we conclude that sporadic ALS mimicking SPMA is present.
K Tsuchiya; S Shintani; M Kikuchi; H Kondo; T Kamaya; S Ohbu; S Kato; H Hayashi; K Ikeda; I Nakano
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Publication Detail:
Type:  Case Reports; Comparative Study; Journal Article    
Journal Detail:
Title:  Journal of the neurological sciences     Volume:  162     ISSN:  0022-510X     ISO Abbreviation:  J. Neurol. Sci.     Publication Date:  1999 Jan 
Date Detail:
Created Date:  1999-06-22     Completed Date:  1999-06-22     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0375403     Medline TA:  J Neurol Sci     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  174-8     Citation Subset:  IM    
Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital, Tokyo Institute of Psychiatry, Japan.
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MeSH Terms
Amyotrophic Lateral Sclerosis / pathology*
Chronic Disease
Diagnosis, Differential
Middle Aged
Muscular Atrophy, Spinal / pathology*

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