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NEUROLOGY 1981;31:1397
© 1981 American Academy of Neurology

Adult GM2 gangliosidosis in association with Tay-Sachs disease

A new phenotype

Ruth Navon, Zohar Argov, Natan Brand and Uri Sandbank

Department of Human Genetics (Dr. Navon), Sackler School of Medicine, Tel-Aviv University. The Chaim Sheba Medical Center, Tel-Hashomer, Department of Neurology. Neuromuscular Clinic (Dr. Argov), Hadassah University Hospital, Jerusalem, Department of Pediatrics (Dr. Brand), The Chaim Sheba Medical Center; and the Department of Pathology Dr. Sandbank. Beilimson Hospital. Petach-Tikvah. Israel.

Address correspondence and reprint requests to Dr. Navon, Institute of Genetics, The Sheba Medical Center, Tel-Hashomer. Israel

Four members of a family had an adult form of hexosaminidase A deficiency. The clinical picture was predominated by selective proximal muscle weakness in the legs and stuttering speech. Two of these patients had children with Tay-Sachs disease.




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