Ubiquitin-immunoreactive inclusions in anterior horn cells and hypoglossal neurons in a case with Joseph's disease.
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.
Suenaga
T
T
Department of Neurology, Faculty of Medicine, Kyoto University, Japan.
Matsushima
H
H
Nakamura
S
S
Akiguchi
I
I
Kimura
J
J
D000328:Adult; D001921:Brain / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D007002:Hypoglossal Nerve / Q000473:pathology / Q000503:physiopathology*; D007150:Immunohistochemistry / Q000379:methods; D002479:Inclusion Bodies / Q000378:metabolism*; D008297:Male; D013116:Spinal Cord / Q000378:metabolism* / Q000473:pathology; D013132:Spinocerebellar Degenerations / Q000378:metabolism* / Q000473:pathology; D013194:Staining and Labeling; D014452:Ubiquitins / Q000378:metabolism*
D014452:Ubiquitins