Angiomatosis, Diffuse Corticomeningeal, Of Divry And Van Bogaert

Clinical Features

Divry and Van Bogaert (1946) described brothers who presented with epilepsy, pseudobulbar syndrome, extrapyramidal signs, dementia, hemianopsia, and 'marbled skin' resulting from a telangiectatic network. Brain examination showed corticomeningeal angiomatosis and myelination of the white substance of the centrum ovale.

Martin et al. (1973) studied 2 sibs, a male and a female, who demonstrated this condition. Both presented similar symptoms of epileptic seizures during the second decade, visual field defects, migraines with focal paresthesias, mental disturbances and progressive dementia. Surgery to relieve these symptoms was unsuccessful. Necropsy revealed diffuse capillarovenous noncalcifying leptomeningeal angiomatoses in the depths of the sulci, becoming more prominent toward the occipital lobes. All the abnormally proliferated vessels showed fibrotic changes. The brain showed diffuse anoxic cortical encephalopathy with areas of atrophy and secondary degeneration of the white matter. These changes became most severe in the parietal-occipital-temporal areas. The brainstem showed signs of fibrillary gliosis of some nuclei and tracts, particularly the vestibular and reticular nuclei, trigeminal spinal tracts, and pyramidal tracts. These patients had no other physical abnormalities or marbling of the skin.

Bussone et al. (1984) reported a 30-year-old man who developed generalized seizures at age 18 years. Over the following several years, he had progressive mental deterioration and developed dysarthria and writing difficulties. He also had hypertension and a deficit of the right lower facial nerve. Brain imaging showed dilatation of the ventricles and diffuse hypodensity of the white matter. Skin findings included a cutaneous venous pattern and cutis marmorata. The disorder was progressive, with the patient developing truncal ataxia, mood instability, and poor cooperation. Angiography showed tapering of the distal branches of the carotid arteries and cerebellar vessels, as well as changes in the meningeal branches. Skin biopsy of external abdominal vessels only showed mild defects of the zonulae occludens between endothelial cells. There was no evidence of calcification.