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Familial progressive neuronal disease and chronic idiopathic intestinal pseudo-obstruction

Departments of Neurology (Drs. Steiner and Argov) and Gastroenterology (Dr. Fich), Hadassah University Hospital and Hebrew University Hadaseah Medical School; and the Departments of Pediatrics (Drs. Steinberg and Faber) and Electrophysiology (Dr. Gilai), Shaare Zedek Medical Center, Jerusalem, Israel.

Chronic idiopathic intestinal pseudo-obstruction (CIIP) is characterized by recurrent episodes of bowel obstruction without mechanical cause. In five members of two Jewish-Iranian families, CIIP was associated with progressive neuronal disease, starting before age 30, with ophthalmoplegia, sensorimotor peripheral neuropathy, and hearing loss. There was no evidence of CNS involvement. The pattern suggested autosomal recessive inheritance.

Address correspondence and reprint requests to Dr. Skiner, Department of Neurology. Hadassah University Hospital, Jerusalem, Israel 91120.

Received June 20, 1986. Accepted for publication in final form October 7, 1986.

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