[Chorea as the presenting form of progressive systemic sclerosis].

We present an exceptional case of a patient with hemichorea of ischemic origin as a form of presentation of progressive systemic sclerosis. The neurologic complications, both central and peripheral, of this condition are considered infrequent and may be due to coexisting or iatrogenic factors, or may be a direct complication of progressive systemic sclerosis. There have been occasional reports of cerebrovascular disease in progressive systemic sclerosis, and this apparent rarity may be related to structural and morphological variations in brain arteries. Recently, there have been reports of primary cerebrovascular changes and vascular calcifications in progressive systemic sclerosis. There are no reported cases associated with movement disorders. The patient is a 66-year-old ex-smoker, and no other vascular risk factors, with chronic Raynaud's phenomenon affecting both hands, and polyarthralgias. Three months previously he began to complain of choreic movements affecting the right-sided limbs without any other neurological manifestations. The abnormal movements responded to haloperidol. There were skin changes suggestive of progressive systemic sclerosis, and capillaroscopy showed abundant avascular zones with dilated capillary loops. Anticentromere antibody titer was elevated to 1/1280. Brain arteriography was normal, and the brain SPECT showed bilateral hypoperfusion bilaterally, predominantly on the left side. Brain MR imaging confirmed an infarct in the left caudate and adjacent internal capsule. The study of this case supports the hypothesis of a direct primary cerebrovascular damage in progressive systemic sclerosis.