Scleroderma (progressive systemic sclerosis) is a multisystem disease affecting the skin, lungs, kidneys, vascular system, myocardium, nervous system, and gastrointestinal tract. Most case reports and texts describing the effects of the disorder
on the nervous system have focused on the peripheral nervous system and the cranial nerves.
Central nervous system (CNS) dysfunction secondary to the vascular effects of scleroderma is recognized and considered a rare manifestation of the disorder. 1-3 When cerebral ischemia is a manifestation of scleroderma, it is generally associated with evidence of renal failure and severe hypertension.4-5 We describe a patient with CREST syndrome (a variant of scleroderma characterized by calcinosis cutis, Raynaud's phenomenon, esophageal reflux, sclerodactyly, and telangiectasia) and CNS vasculitis who was treated with high-dose immunosuppressive therapy, resulting in dramatic clinical improvement.