Olivopontocerebellar degeneration. Clinical and ultrastructural abnormalities

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

Research output: Contribution to journalArticle

69 Citations (Scopus)

Abstract

Thirty three persons in the Schut Swier kindred at risk for autosomal dominant olivopontocerebellar degeneration were 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
StatePublished - 1974

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Olivopontocerebellar Atrophies
Biopsy
Nucleocapsid
Purkinje Cells
Motor Neurons
Degeneration
Cells

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Landis, D. M. D., Rosenberg, R. N., Landis, S. C., Schut, L., & Nyhan, W. L. (1974). Olivopontocerebellar degeneration. Clinical and ultrastructural abnormalities. Archives of Neurology, 31(5), 295-307.

Olivopontocerebellar degeneration. Clinical and ultrastructural abnormalities. / Landis, D. M D; Rosenberg, R. N.; Landis, S. C.; Schut, L.; Nyhan, W. L.

In: Archives of Neurology, Vol. 31, No. 5, 1974, p. 295-307.

Research output: Contribution to journalArticle

Landis, DMD, Rosenberg, RN, Landis, SC, Schut, L & Nyhan, WL 1974, 'Olivopontocerebellar degeneration. Clinical and ultrastructural abnormalities', Archives of Neurology, vol. 31, no. 5, pp. 295-307.
Landis, D. M D ; Rosenberg, R. N. ; Landis, S. C. ; Schut, L. ; Nyhan, W. L. / Olivopontocerebellar degeneration. Clinical and ultrastructural abnormalities. In: Archives of Neurology. 1974 ; Vol. 31, No. 5. pp. 295-307.
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