@article{oai:niigata-u.repo.nii.ac.jp:00024384,
 author = {内藤, 明彦},
 issue = {12},
 journal = {新潟医学会雑誌, 新潟医学会雑誌},
 month = {Dec},
 note = {An entity of dentatorubropallidoluysian atrophy（DRPLA）are based upon its major neuropathologic findings consisting of combined degeneration of the dentatorubral and pallidoluysian systems. DRPLA can display a variety of symptoms, including myoclonus epilepsy syndrome, cerebellar ataxia, and choreoathetosis or chorea, and is inherited as autosomal dominant traits. Differential diagnosis is discussed. Joseph disease can be differentiated from DRPLA by the following two characteristics; （1）neither epileptic seizures nor dementia, （2）additional, but specific degeneration in the substantia nigra, pontine nucleus, cranial nerve nuclei, and the spinal cord. Mitochondorial encephalomyopathy （MERRF type）also can be distinguished from DRPLA by the following four points; （1）ragged-red-fibers in skeletal muscles, （2）increase of lactic and pyruvic acid level in blood and cerebrospinal fluid, （3）additional degenerative changes in the spinal cord, （4）inheritance pattern only through maternal side.},
 pages = {733--738},
 title = {5) 歯状核・赤核・淡蒼球・ルイ体萎縮症（内藤・小柳型）(シンポジウム 遺伝性脊髄小脳変性症 : 最近の知見から, 第417回新潟医学会)},
 volume = {100},
 year = {1986}
}