CEREBRAL AUTOSOMAL-DOMINANT ARTERIOPATHY WITH SUBCORTICAL INFARCTS AND LEUKOENCEPHALOPATHY (CADASIL) - CONFIRMATION BY CEREBRAL BIOPSY IN 2CASES

CADASIL is a recently recognized familial form of subcortical multi-in farct dementia. The pathogenesis of the disease is unknown, but it is characterized pathologically by a novel vasculopathy affecting leptome ningeal and subcortical arteries. We describe 2 cases in which the dia gnosis could be made from the cerebral biopsy appearances of affected leptomeningeal vessels. The ultrastructural appearances of the vessel wall deposit are illustrated, and their similarity to those of immune complex deposits discussed.