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Clinico-radiological profile of childhood moyamoya disease - a study of 30 children from India

      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.