UNE MALADIE FAMILIALE CARACTERISEE PAR UNE ANGIOMATOSE DIFFUSE CORTICO-MENINGEE NON CALCIFIANTE ET UNE DEMYELINISATION PROGRESSIVE DE LA SUBSTANCE BLANCHE

Abstract

Clinical history, evolution of the disease and extensive clinical examination in 2 brothers are described together with a postmortem examination of the brain in one of the cases. An extensive family history is included, there being 3 brothers afflicted in a family of 6 boys and 3 girls. The condition is characterized by convulsive seizures, mental deterioration and final neurologic deficites with onset at about 18-24 yrs. The combination of angio-matous and demyelinating lesions are described in detail with very adequate neuropathologic studies. A consider-able review and discussion of related syndromes are included.