Familial Capillaro-Venous Leptomeningeal Angiomatosis

Abstract

Two siblings presented a similar disorder characterized by the following features: onset during the second decade or at the beginning of the third, migrainous attacks with focal paresthesias, Jacksonian epileptic fits with postictal paralysis, temporal and grand mal seizures, repeated status epilepticus, focal deficits, visual field defects, aphasic and apraxic disturbances, and a progressive dementia. In both cases, repeated arteriograms suggested the possibility of vascular malformation; a right temporal cavernoma was also discovered in one patient. Both siblings were operated on with unsatisfactory results. Post-mortem studies gave basically similar results: (1) diffuse capillaro-venous noncalcifying leptomeningeal angiomatosis increasing towards the occipital lobes; (2) focal and diffuse, old and fresh cortical anoxic damage causing cortical atrophy with secondary degeneration of the white matter, reinforced in the parietotemporo occipital areas; and (3) fibrillary gliosis selectively involving some nuclei and tracts in the brain stem. Minor differences were found and are discussed in the text. Examination of the viscera failed to reveal any congenital malformation or tumor. The clinical features and the nosological situation of this familial angiomatosis are discussed. The disease, in our cases, is obviously similar to the syndrome of Di-vry and van Bogaert although certain differences are pointed out.

Vascular malformations of the central nervous system: A morphological overview

“…Related conditions include diffuse familial capillarovenous leptomeningeal angiomatosis or angiodysplasia in infants with secondary vascular brain damage [160], and Divry-Van Bogaert-syndrome, a noncalcifying diffuse corticomeningeal angiomatosis with sudanophilic white matter changes occasionally referred to as sudanophilic leukodystrophy [57,111].…”