"Angiodysgenetic necrotizing encephalopathy" and its different manifestations. Survey of age-related forms and clinico-pathological appearances

Abstract

Various observations of diffuse meningo-cerebral angiomatoses, which cannot be satisfactorily classified with the common phakomatoses, have been reported. They may occur at any age, with familial accumulation or sporadically. Divry and van Bogaert were the first to draw attention to such conditions in adults, where meningo-cerebral angiomatoses seemed to be combined with sudanophilic leukodystrophy. However, subsequently the latter was considered to be due to hypoxic damage to the white matter. In other observations, the severe damage to the grey matter was more evidently of hypoxic origin. Observations on two newborn individuals, sporadic examples of diffuse meningo-cerebral angiomatosis and with severe necrotic changes in the grey and white matter, are reported and discussed. Published reports on the various age-related forms are summarized and a general designation is suggested, which includes the various observations under a general heading. A parallel will be drawn between the meningo-cerebral angiomatosis and Foix-Alajouanin's disease.