BRAIN INVOLVEMENT IN SCLERODERMA - 2 AUTOPSY CASES

Background-Neuropathological data are very scarce in systemic sclerosi s and fail to demonstrate primary changes in the brains of such patien ts. Case Descriptions-A 41-year-old woman with CREST syndrome develope d signs of dementia after an episode of severe dehydration and died tw o months later of septic shock. A 63-year-old woman with CREST syndrom e and a history of two unexplained transient ischemic attacks had had balance disorders since age 62. She died of severe pulmonary hypertens ion. In both cases, the autopsy showed extensive wall calcification of small arteries and arterioles in the brain, primarily in the basal ga nglia, and also in the frontal lobes and the cerebellar area in the se cond case. No known cause of cerebrovascular calcification was found i n either patient. Conclusion-The neuropathological findings in these t wo patients suggest that systemic sclerosis may induce primary vascula r changes in the brain, of which calcification may be a marker.