Nigro-spino-dentatal degeneration with nuclear ophthalmoplegia. A unique and partially treatable clinico-pathological entity

Bryan T. Woods, Herbert H. Schaumburg

Research output: Contribution to journalArticle

163 Scopus citations

Abstract

A family with a dominant form of neuronal degeneration, marked clinically by ataxia, hyperreflexia, distal motor weakness, extrapyramidal rigidity, bulbar signs, and ophthalmoplegia has been followed for some years. Recent post-mortem study of 1 member revealed degeneration of the anterior horn cells, spino-cerebellar tracts, pons, dentate nucleus, substantia nigra, and oculomotor nuclei. This pattern of involvement appears to be a unique variant of neuronal degeneration, sharing features of both spino-cerebellar and extra-pyramidal system degenerations. Furthermore, the extrapyramidal signs of 3 affected family members have responded to a combination of dopaminergic and central anti-cholinergic medications; levodopa, amantidine hydrochloride, and trihexyphenidyl hydrochloride.

Original languageEnglish (US)
Pages (from-to)149-166
Number of pages18
JournalJournal of the Neurological Sciences
Volume17
Issue number2
DOIs
StatePublished - Oct 1972

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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