Intracranial Aneurysms in Patients With CREST Syndrome -Two Case Reports-

Abstract

CREST syndrome is a variant of scleroderma characterized by calcinosis, Raynaud's phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia, and is a collagen vascular disease characterized by inflammation and fibrosis of multiple organs/tissues. Neurological and cerebrovascular abnormalities are uncommon in CREST syndrome. Here, we report two patients with CREST syndrome harboring intracranial aneurysms. A 53-year-old woman with a 6-month history of CREST syndrome had multiple intracranial aneurysms that arose from the right middle cerebral artery, the left middle cerebral artery, the choroidal segment of the left internal carotid artery, and the left anterior cerebral artery. A 64-year-old woman with a 2-year history of CREST syndrome had a fusiform aneurysm located on the insular segment of the left middle cerebral artery. These patients were treated surgically and good outcome was achieved in both cases. The pathogenesis of cerebral aneurysms associated with collagen diseases, including CREST syndrome, remains unclear. Early treatment of CREST syndrome and other collagen diseases may prevent arteritis from progressing to affect the intracranial arteries and thus reduce the occurrence of aneurysms. The prognosis for patients with collagen diseases after rupture of cerebral aneurysm seems to be poor because the multiplicity, atypical morphology, and atypical location of their aneurysms make treatment difficult. Thus, early detection and treatment are important to improve the prognosis.

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“…En los años 60, Carr et al y Schimke et al describieron el síndrome CREST como una variante de la esclerodermia sistémica, siendo más limitada, indolente y caracterizada por calcinosis, fenómeno de Raynaud, alteración de la motilidad esofágica, esclerodactilia y telangiectasias 1 . Normalmente, sólo tres de estas cinco características son necesarias para establecer el diagnóstico 2 . De una manera general, el sexo femenino es el más afectado 1,3,4 , en una proporción de 3:1.…”