Familial progressive neuronal disease and chronic idiopathic intestinal pseudo-obstruction
Steiner, I.; Steinberg, A.; Argov, Z.; Faber, J.; Fich, A.; Gilai, A.
Neurology 37(6): 1046-1050
1987
ISSN/ISBN: 0028-3878 PMID: 3035426 Document Number: 301997
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.