Introduction: Childhood moyamoya disease, a vaso-occlusive disease, has myriad presentations. Early diagnosis is key to treatment and successful outcome.
Methods: 30 patients of childhood moyamoya disease diagnosed by MRI, MR-Angiography and DSA, were studied for various spectrum of clinico-radiological manifestations.
(Appropriate approval/consent taken)
Results: Mean age: 6.71 yrs (6 months – 15 years); Female:Male ratio 1.15:1.
Clinically, headache, cognitive decline, bihemispherical TIAs, visual loss and radiologically, Boomerang sign, unilateral affection and posterior circulation involvement were the unusual presentations found in this study.
Conclusions: Knowledge of varied manifestations must be borne in mind for early diagnosis to achieve favorable prognosis of this potentially treatable entity.
© 2015 Published by Elsevier Inc.