The 80-year history of the oculopharyngeal syndrome (ptosis familiaris hereditaria tarda) is summarized together with the author’s own experience of incidence in three generations of a Hungarian family. This is the 19th verified case of oculopharyngeal syndrome (OP sy.) in the literature and the 14th in which hereditary familial incidence could be confirmed. This study covered five generations and a total of 64 persons, of which 61 could be examined. Eight members of the family were affected by the disorder, five of them being examined personally by the author. In every case, the onset of the symptoms occurred between 45 and 50 years of age, only females were affected, and there was no other muscular impairment apart from ptosis and dysphagia. The disease progressed slowly or occasionally remained in a stationary state. Some constitutional abnormalities were also observed as accessory symptoms. They are considered to play a predisposing role and probably promote the progression of dysphagia as ptosis develops. The disease has a hereditary familial nature and its inheritance is dominant. On the basis of its typical symptoms (course, onset, hereditary nature and well-definable histopathology), OP sy. can be regarded as an independent disease entity within the group of progressive muscular dystrophies.

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