ISSN:
1432-0533
Keywords:
Joseph's disease
;
Ubiquitin
;
Immunocytochemistry
;
Anterior horn cell
;
Hypoglossal nucleus
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary We describe a patient with progressive spastic ataxia and ophthalmoparesis. His clinical and neuropathological findings were consistent with Joseph's disease. The most characteristic neuropathological features in the present case were ubiquitin-immunoreactive filamentous or dense inclusions in spinal anterior horn cells and hypoglossal neurons, which have been considered to be a specific finding in amyotrophic lateral sclerosis (ALS). The occurrence of ubiquitin-immunoreactive inclusions suggests that such inclusions are not totally specific to ALS and could occur in occasional degenerating motor neurons without apparent ALS neuropathology.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00227732
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