Dentatorubro-pallidoluysian degeneration: Clinical, neuro-ophthalmologic, biochemical, and pathologic studies on autosomal dominant form

Ikuo Goto, Shozo Tobimatsu, Michiya Ohta, Shinichi Hosokawa, Hiroshi Shibasaki, Yoshigoro Kuroiwa

Research output: Contribution to journalArticle

38 Citations (Scopus)

Abstract

Four cases in two generations of a Japanese family are described as affected by an autosomal dominant-inherited cerebellar ataxia, pyramidal and extrapyramidal signs, and abnormal ocular movements, resembling Machado-Joseph disease. The neuro-ophthalmologic examination suggests the involvement of multiple motor control systems. The neuropathologic examination of one case showed neuronal loss in the subthalamic nuclei, pallidum, dentate nuclei of cerebellum, and red nuclei, which is distinct from Machado-Joseph disease.

Original languageEnglish
Pages (from-to)1395-1399
Number of pages5
JournalNeurology
Volume32
Issue number12
DOIs
Publication statusPublished - Dec 1982

All Science Journal Classification (ASJC) codes

  • Clinical Neurology

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