Abstract

Hereditary spherocytosis (HS) is due to alterations in genes that encode for proteins such as spectrin and ankyrin involved in vertical associations that tie the membrane skeleton to the lipid bilayer. Moya Moya disease is defined as severe bilateral stenosis of the arteries around circle of Willis with prominent collateral circulation. Hereditary spherocytosis causes decreased deformability of RBCs. Decreased deformability of RBC along with increased blood flow associated with anemia contribute to endothelial proliferation which progresses slowly and produces stenosis followed by occlusion. Here we present a young patient presenting with stroke who was diagnosed with hereditary spherocytosis associated with Moya Moya disease. Consider the possibility of Moya Moya disease in patient with HS presenting with stroke.

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