An Autopsied Case of Dentatorubropallidoluysian Atrophy with Atypical Pathological Features
Corresponding Author
Katsuyoshi Mizukami M.D
Department of Psychiatry, Institute of Clinical Medicine, University of Tsukuba, Tsukuba
Department of Psychiatry, Institute of Clinical Medicine, University of Tsukuba, 1–1–1 Tennodai, Tsukuba, Ibaraki 305, Japan.Search for more papers by this authorMegumi Sasaki M.D
Department of Psychiatry, Institute of Clinical Medicine, University of Tsukuba, Tsukuba
Search for more papers by this authorHiroyasu Shiraishi M.D
Department of Psychiatry, Institute of Clinical Medicine, University of Tsukuba, Tsukuba
Search for more papers by this authorJunzo Koizumi M.D
Department of Psychiatry, Institute of Clinical Medicine, University of Tsukuba, Tsukuba
Search for more papers by this authorTakesaburo Ogata M.D
*Department of Pathology, Institute of Basic Medical Sciences, University of Tsukuba, Tsukuba
Search for more papers by this authorKenji Kosaka
**Department of Psychiatry and Neurology, Yokohama City University, School of Medicine, Yokohama
Search for more papers by this authorCorresponding Author
Katsuyoshi Mizukami M.D
Department of Psychiatry, Institute of Clinical Medicine, University of Tsukuba, Tsukuba
Department of Psychiatry, Institute of Clinical Medicine, University of Tsukuba, 1–1–1 Tennodai, Tsukuba, Ibaraki 305, Japan.Search for more papers by this authorMegumi Sasaki M.D
Department of Psychiatry, Institute of Clinical Medicine, University of Tsukuba, Tsukuba
Search for more papers by this authorHiroyasu Shiraishi M.D
Department of Psychiatry, Institute of Clinical Medicine, University of Tsukuba, Tsukuba
Search for more papers by this authorJunzo Koizumi M.D
Department of Psychiatry, Institute of Clinical Medicine, University of Tsukuba, Tsukuba
Search for more papers by this authorTakesaburo Ogata M.D
*Department of Pathology, Institute of Basic Medical Sciences, University of Tsukuba, Tsukuba
Search for more papers by this authorKenji Kosaka
**Department of Psychiatry and Neurology, Yokohama City University, School of Medicine, Yokohama
Search for more papers by this authorAbstract
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.
References
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