title: Ubiquitin-immunoreactive inclusions in anterior horn cells and hypoglossal neurons in a case with Joseph's disease.
abstract: 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.
authors.affiliation: Department of Neurology, Faculty of Medicine, Kyoto University, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Suenaga
authors.forename: H
authors.initials: H
authors.lastname: Matsushima
authors.forename: S
authors.initials: S
authors.lastname: Nakamura
authors.forename: I
authors.initials: I
authors.lastname: Akiguchi
authors.forename: J
authors.initials: J
authors.lastname: Kimura
mesh_terms: 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*
chemical_list: D014452:Ubiquitins