Abstract

PROENCA, Lúcia et al. Moyamoya Syndrome and Sickle Cell Anemia: A Clinical Report. Medicina Interna [online]. 2017, vol.24, n.2, pp.119-123. ISSN 0872-671X.

moyamoya syndrome is a rare clinical entity of unkown etiology which is described in association with various diseases, including sickle cell anemia. The authors present a case of a 28-year-old woman with known history of sickle-cell anemia, two ischemic stroke episodes and long term hemiparesis, seeks the emergency department due to worsening of the left hemiparesis, with homolateral paresthesias and fronto-parietal headache. The physical examination confirmed the neurologic deficit. Brain computed tomographyand angio-magnetic resonance imaging showed vascular abnormalities, with bilateral internal carotid and medial cerebral artery stenosis and small and entangled sublenticular vessels, suggesting the moyamoya-like pattern. A diagnostic cerebral angiography was performed, which confirmed this abnormal vascular pattern. Conservative medical treatment was the option in this particular case, taking into account the risks involved in an invasive surgical procedure. “moyamoya” means, in japanese, “puff of smoke”, illustrating this a typical angiographic vascular pathologic pattern. We intend not only to describe this clinical case, but also to review the current evidence about this rare clinical entity.

Keywords : Anemia, Sickle Cell; moyamoya Disease; Sickle Cell Trait; Stroke.

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