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Numerous bleeding episodes, neurological signs and symptoms, and pulmonary arteriovenous malformations are well documented in reports of families with hereditary hemorrhagic telangiectasia. It is always assumed that these are due to the arteriovenous malformations. We are here reporting a case with positive cerebral angiographic findings of large arteriovenous communications, as well as catheterization data revealing pulmonary abnormalities secondary to the cerebral arteriovenous fistula, but no abnormalities within the pulmonary vasculature itself.

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Abstract. A clinical analysis of three members of a family with documented osteopetrosis and familial paraplegia is presented. All patients had a long history of increased bone density and slowly progressing paraparesis of both legs. A thorough review of the literature has revealed no other cases which presented with paraplegia without spinal cord com pression. Although the etiologic factor or factors remain unknown, our review supports the contention that this is a distinct clinical entity.