Spatz-Lindenberg disease - A rare cause of vascular dementia

Background-Isolated cerebral. thromboangiitis obliterans (Spatz-Lindenberg disease) is not well recognized as a cause of vascular dementia. Case Description-A 58-year-old woman presented with dementia and pyramidal signs. Neuroimaging showed multiple areas of white matter change. Brain bio psy showed intimal thickening of the walls of leptomeningeal and intraparen chymal arteries, almost to complete occlusion, with an intact internal elas tic lamina and media and without inflammation or infiltration. The cortex s howed only moderate gliosis. Conclusions-Spatz-Lindenberg disease should be considered in the differenti al diagnosis of vascular dementia. Additional studies of its pathogenesis a re required to determine appropriate treatment.