Research Article| Volume 64, ISSUE 1, P33-43, April 1984

“Rimmed vacuole myopathy” sparing the quadriceps

A unique disorder in iranian jews
  • Z. Argov
    Correspondence to: Z. Argov, M.D., Department of Neurology, Hadassah University Hospital, P.O. Box 12 000, Jerusalem, 91120 Israel.
    Department of Neurology, Hadassah University Hospital and Hebrew University-Hadassah Medical School, Jerusalem Israel
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  • R. Yarom
    Department of Pathology, Hadassah University Hospital and Hebrew University-Hadassah Medical School, Jerusalem Israel
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      Nine cases from 4 different Iranian-Jewish families presented with generalized muscular weakness. Quadriceps muscle was the only leg muscle which retained its normal power and function even in advanced cases. Biopsies from severely involved muscles revealed a typical “rimmed vacuole myopathy” while the quadriceps and deltoid showed minimal changes. CK levels were normal or moderately elevated and the EMG pattern suggested an underlining neurogenic disorder. The possible neurogenic character of this autosomal recessive disorder is discussed with relevance to the rimmed vacuoles.


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