Brain Involvement in Scleroderma

Abstract

Background-Neuropathological data are very scarce in systemic sclerosis and fail to demonstrate primary changes in the brains of such patients. Case Descriptions-A 41-year-old woman with CREST syndrome developed signs of dementia after an episode of severe dehydration and died two months later of septic shock. A 63-year-old woman with CREST syndrome and a history of two unexplained transient ischemic attacks had had balance disorders since age 62. She died of severe pulmonary hypertension. In both cases, the autopsy showed extensive wall calcification of small arteries and arterioles in the brain, primarily in the basal ganglia, and also in the frontal lobes and the cerebellar area in the second case. No known cause of cerebrovascular calcification was found in either patient. Conclusion-The neuropathological findings in these two patients suggest that systemic sclerosis may induce primary vascular changes in the brain, of which calcification may be a marker. (Stroke. 1998;29:719-721.)

Noninflammatory Cerebral Vasculopathy Associated with Recurrent Ischemic Strokes

“…Dermal sclerosis, as demonstrated on skin biopsy, has been associated with systemic sclerosis 11 ; however, neuropathological investigations in this condition have revealed calcification or necrosis rather than collagen deposition. 12,13 Our patient had none of the clinical features of focal dermal or systemic sclerosis, and biopsy revealed no evidence of calcification or necrosis, to suggest this as an underlying etiology. Periventricular venous collagenosis (PVC) has been described in elderly patients with leukoariosis but not stroke.…”

Intracerebral Calcification in Systemic Sclerosis

“…13 Intracerebral calcification might result from necrosis of smooth muscle cells of the small cerebral arteries, which contain large amounts of calcium that could therefore be trapped and accumulate in the arterial wall. Cerebrovascular involvement often seems quiescent in scleroderma but might contribute to the onset of CNS disorders during low-flow states, as described in 1 patient, 3 or via vasospastic phenomena, revealed by serial angiography in a case report. 10 Increased vasospasm has been demonstrated in the main internal organs (heart, kidney, and lung) in scleroderma 11 and might explain why some middle-aged scleroderma patients have otherwise unexplained transient ischemic attacks with focal neurological defects 3,14,15 or transient global amnesia, 16 both of which were observed in our patients.…”

“…Cerebrovascular involvement often seems quiescent in scleroderma but might contribute to the onset of CNS disorders during low-flow states, as described in 1 patient, 3 or via vasospastic phenomena, revealed by serial angiography in a case report. 10 Increased vasospasm has been demonstrated in the main internal organs (heart, kidney, and lung) in scleroderma 11 and might explain why some middle-aged scleroderma patients have otherwise unexplained transient ischemic attacks with focal neurological defects 3,14,15 or transient global amnesia, 16 both of which were observed in our patients. In addition, various psychological disorders have been reported in scleroderma patients 15,[17][18][19] and may be partly due to organic brain involvement; indeed, it has recently been shown that patients with basal ganglia calcification frequently have neuropsychological alterations.…”

“…2 We recently described 2 patients with scleroderma, central nervous system (CNS) manifestations, and autopsy evidence of small cerebral artery wall calcification, mainly in the basal ganglia. 3 Both patients also had mild basal ganglia calcification on brain CT, which is the most frequently observed CT abnormality in the brain of patients with cerebral lupus. 4 Because of the young age of the 2 patients and the absence of other known causes of cerebrovascular calcification, we postulated that the latter were primarily related to scleroderma.…”

“…4 Because of the young age of the 2 patients and the absence of other known causes of cerebrovascular calcification, we postulated that the latter were primarily related to scleroderma. 3 To confirm this we conducted a prospective CT study to detect intracerebral calcification in patients with systemic sclerosis. …”