Olivopontocerebellar Degeneration: Clinical and Ultrastructural Abnormalities

D. M D Landis, R. N. Rosenberg, S. C. Landis, L. Schut, W. L. Nyhan

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Abstract

Thirty-three persons in the Schut-Swier kindred at risk for autosomal dominant olivopontocerebellar degeneration have been examined and nine new cases identified. These affected individuals manifested severe cerebellar deficits; abnormalities of cortical, bulbar, and spinal motor neurons; and posterior column dysfunction. Cerebellar biopsy material from two patients revealed extensive progressing loss of Purkinje cells, degeneration of cortical afferents, and variable loss of granule cells. Vermiform tubules that resemble paramyxovirus nucleocapsids were found in association with crystalline inclusions in both biopsy specimens.

Original languageEnglish (US)
Pages (from-to)295-307
Number of pages13
JournalArchives of neurology
Volume31
Issue number5
DOIs
StatePublished - Nov 1974

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ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

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