Divry- Van Bogaert Syndrome

Synonyms: familial type orthochromatic leukodystrophy with diffuse leptomeningeal angiomatosis, sudanophilic leuokodystrophy with meningeal angiomatosis, infantile form of above.

In adults it affects men with deep and superficial strokes with corticomeningeal noncalcifying angiomatosis, dementia, livedo reticularis, seizures, and pseudobulbar palsy presenting between ages 18-40 with death within two decades. Some have called it "Binswanger's with LR." It can be familial or sporadic. Most patients have stiff or slow movements and gait, altered speech, rigidity, ataxia and hyperreflexia. Path, U fibers are spared. EM shows splitting of vascular basal membrane and electron dense lysosome like entities and hypertrophic pericytes.

The infantile form is usually familial, can affect girls also, and is not associated with LR.