Objectives: To observe the common clinical presentation & its association with neuroimaging abnormality.

Methods: This cross-sectional study was conducted among children under 18 yrs & moyamoya was diagnosed on the basis of RCMJ criteria. Their clinical and neuroimaging findings were analyzed.

Results: 20 children diagnosed as moyamoya disease were enrolled. The mean age was 7.24 ± 3.34 (3-14) years. Male: Female ratio was 1.2:1.The majority (n=18) had ischaemic stroke.

16 (80%) exhibited hemiparesis and 4 had alternating hemiparesis. The other presentations were dysarthria (n=13), visual impairment and convulsion (5 each), headache, altered sensorium, ataxia, involuntary movements in each of 3 cases, papilloeda and abnormal vital signs in each of 2 cases. Among presenting features, convulsions, altered sensorium, alternating hemiplegia, papilloeda and abnormal vital signs were significantly (p < 0.5) associated with abnormal CT scan.

Stroke risk factors or FHO stroke in 5/20 (p<0.3) and previous experience of TIA in 9/20 (p<.01) were strongly associated with subsequent occurrence of stroke in under and over 5 yrs children respectively.“Puff of smoke” appearance on MRA was present in 19 cases. One case was diagnosed through cerebral DSA.Bilateral ICA and MCA stenosis was most commonly observed abnormality followed by ACA and PCA.

Conclusion: Most cases presented with ischaemic stroke. About half of the cases previously experienced TIA, which foretells the evaluation for moyamoya through angiogram. Other clinical features correlated with the presentations of moyamoya reported elsewhere.