Moyamoya Syndrome and Sickle Cell Anemia: A Clinical Report

Abstract

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 frontoparietal
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.