Introduction: Moyamoya disease is an uncommon cerebrovascular disease characterized by progressive steno-occlusive changes in terminal internal carotid arteries (ICAs) and their main branches, associated with development of moyamoya vessels.1 We present the largest case series of childhood moyamoya disease from India.
Materials and methods: Thirty patients of childhood Moyamoya disease, whose diagnosis was confirmed by magnetic resonance imaging (MRI), MR angiography (MRA), and digital subtraction angiography (DSA), were studied for various spectrum of clinical and radiological manifestations.
Results: Of the 30 patients evaluated (mean age: 6.71 years; F:M ratio 1.15:1), majority (96.66%) presented with ischemic symptoms, whereas only one (3.33%) had hemorrhagic stroke. Ischemic stroke with hemiparesis was the most common presenting feature (66.66%); rarer manifestations of moyamoya disease like headache, seizures, cognitive decline, visual loss, and bihemispherical transient ischemic attacks (TIAs) were also seen as presenting features. Radiologically, other than ischemic and hemorrhagic stroke, normal parenchyma with abnormal flow voids was the uncommon pattern seen on MRI brain, seen in 23.33%; 1 patient had isolated corpus callosal infarct. On angiography, 21 (70%) had bilateral ICA disease, 5 (16.66%) had unilateral ICA disease, and 4 (13.33%) had bilateral ICA with associated posterior circulation involvement.
Conclusion: Other than seizures and strokes, headache, cognitive decline, bihemispherical TIAs and isolated corpus callosal involvement were the unusual presentations found in this study. Radiologically, unilateral affection and posterior circulation involvement were also found. It is important to be familiar with the usual and unusual clinical manifestations and MRI/MRA findings in moyamoya disease to make an early diagnosis leading to a good prognosis.
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