OCULOPHARYNGEAL MUSCULAR-DYSTROPHY - NON-FRENCH-CANADIAN PEDIGREES

Oculopharyngeal muscular dystrophy (OPMD) is a late adult onset, autos omal dominant muscular dystrophy characterized by ptosis and dysphagia . The OPMD gene has been localized to chromosome 14q11.2-q13 in French -Canadian pedigrees. We report 2 non-French-Canadian families with OPM D. Affected ancestors were immigrants to the United States from Italy and Normandy. The Norman pedigree does not share the French-Canadian h aplotype, OPMD appears to be a heterogeneous disorder with similar phe notypes, but probably with different gene loci. (C) 1998 John Wiley & Sons, Inc.