ISSN:
1432-0533
Keywords:
Key words Amyotrophic lateral sclerosis
;
SOD1 gene
;
Posterior column
;
Lewy-body-like inclusion
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract A 65-year-old man with familial amyotrophic lateral sclerosis (ALS) with posterior column involvement showed fairly slow progression of the illness and lived with the aid of a respirator for 12 years. Neuropathological examinations showed simultaneous involvement of the pyramidal tract and lower motor neurons as well as degeneration in the Clarke’s nucleus- spinocerebellar tract- middle root zone of the posterior column, the pallido-luysian system, the medullary reticular formation, and widespread anterolateral columns of the spinal cord. However, the patient had no Lewy-body-like hyaline inclusions, which are characteristic features of this form of familial ALS. Moreover, no abnormalities were found in his SOD1 cDNA sequences. There seem to be certain heterogeneities in familial ALS with posterior column involvement, and SOD1 gene abnormalities may be involved in the pathomechanism in rapidly progressing ALS, in which there are Lewy-body-like hyaline inclusions.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/s004010050557
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