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An Autopsied Case of Dentatorubropallidoluysian Atrophy with Atypical Pathological Features
Author(s) -
Mizukami Katsuyoshi,
Sasaki Megumi,
Shiraishi Hiroyasu,
Koizumi Junzo,
Ogata Takesaburo,
Kosaka Kenji
Publication year - 1992
Publication title -
psychiatry and clinical neurosciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.609
H-Index - 74
eISSN - 1440-1819
pISSN - 1323-1316
DOI - 10.1111/j.1440-1819.1992.tb00551.x
Subject(s) - pathological , atrophy , pathology , medicine , neuroscience , psychology
Abstract: This is a report of an autopsied case of dentatorubropallidoluysian atrophy (DRPLA) with atypical neuropathological Andings. The patients was a 31‐year‐old female. Her clinical symptoms were epileptic seizures, cerebellar ataxia, choreoathetosis and dementia. A neuropathological examination revealed the Abrillary gliosis in various areas of the CNS and severe degeneration in the cerebellar cortex and nucleus fwiculi dorsalis in addition to a marked degeneration of the dentatorubropallidoluysian systems. The present case is diagnosed neuropathologically as DRPLA associated with the flndings of chronic diphenylhydantoin intoxication and epileptic brain damage.