This is a case report of a thirty-seven-year-old man with systemic noninflam matory vasculopathy who developed a slowly progressive dementia with multi ple sites of stroke. Cerebral angiography revealed abnormal terminal vascula ture with beading and occlusions. Brain biopsy confirmed a noninflammatory vascular pathology. We have also reviewed the world literature relevant to this clinicopathologic entity and have discussed its relationship to thromboangiitis obliterans cerebri.
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