NEUROPATHIC FINDINGS IN OCULOPHARYNGEAL MUSCULAR-DYSTROPHY - A REPORTOF 7 CASES AND A REVIEW OF THE LITERATURE

We describe seven patients with clinical evidence of oculopharyngeal m uscular dystrophy. Four of these patients were members of the same Ita lian-American family. The age at onset was after the fourth decade in all patients. All seven patients had extraocular muscle involvement, a nd six of the seven patients had clinical, electrophysiological, and/o r pathological evidence of neuropathy in addition to features that wer e suggestive of myopathy. An autopsy was performed on one patient. We discuss the significance of the concurrence of neuropathic features wi th oculopharyngeal muscular dystrophy in relation to these patients an d previously reported cases.