Neuropathic Findings in Oculopharyngeal Muscular Dystrophy: A Report of Seven Cases and a Review of the Literature

Orla Hardiman, Barbara E. Shapiro, M. A. Farrell, John J. Halperin, Shirley H. Wray, Robert H. Brown

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

We describe seven patients with clinical evidence of oculopharyngeal muscular dystrophy. Four of these patients were members of the same Italian-American family. The age at onset was after the fourth decade in all patients. All seven patients had extraocular muscle involvement, and six of the seven patients had clinical, electrophysiological, and/or pathological evidence of neuropathy in addition to features that were suggestive of myopathy. An autopsy was performed on one patient. We discuss the significance of the concurrence of neuropathic features with oculopharyngeal muscular dystrophy in relation to these patients and previously reported cases.

Original languageEnglish (US)
Pages (from-to)481-488
Number of pages8
JournalArchives of Neurology
Volume50
Issue number5
DOIs
StatePublished - Jan 1 1993
Externally publishedYes

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Oculopharyngeal Muscular Dystrophy
Oculomotor Muscles
Muscular Diseases
Age of Onset
Autopsy

All Science Journal Classification (ASJC) codes

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

Cite this

Hardiman, Orla ; Shapiro, Barbara E. ; Farrell, M. A. ; Halperin, John J. ; Wray, Shirley H. ; Brown, Robert H. / Neuropathic Findings in Oculopharyngeal Muscular Dystrophy : A Report of Seven Cases and a Review of the Literature. In: Archives of Neurology. 1993 ; Vol. 50, No. 5. pp. 481-488.
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Neuropathic Findings in Oculopharyngeal Muscular Dystrophy : A Report of Seven Cases and a Review of the Literature. / Hardiman, Orla; Shapiro, Barbara E.; Farrell, M. A.; Halperin, John J.; Wray, Shirley H.; Brown, Robert H.

In: Archives of Neurology, Vol. 50, No. 5, 01.01.1993, p. 481-488.

Research output: Contribution to journalArticle

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