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Pediatric Moyamoya Disease: Clinical Profile, Literature Review and Sixteen Year Experience from a Tertiary Care Teaching Institute

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Abstract

Objective

To assess the clinical spectrum and outcome of pediatric moyamoya disease (MMD) in Indian sub-continent.

Methods

The authors retrospectively analysed data of 23 patients, diagnosed with pediatric moyamoya disease from a single center during the period of 1996–2011.

Results

There were 18 boys and 5 girls. Mean age at onset of symptoms was 3.8 ± 2.2 y. All patients presented with cerebral ischemic events. Recurrent stroke was the presenting feature in 12(52.2 %) patients. Twenty one patients were definitive case of moyamoya disease and two were probable case of moyamoya disease. Posterior circulation was involved in 26.1 % patients. Three patients underwent indirect surgical revascularisation procedure and rest were managed conservatively. On follow up 66.6 % patients had residual neurological deficit.

Conclusions

Heightened awareness of this entity among pediatric neurologists is required for early diagnosis of pediatric moyamoya disease.

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Contributions

PS and AC designed the study. PS was the consultant in charge of the cases. NK interpreted radiological data. AC wrote the first draft of manuscript and all co-authors contributed in critical review of manuscript writing.

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Correspondence to Pratibha Singhi.

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Singhi, P., Choudhary, A. & Khandelwal, N. Pediatric Moyamoya Disease: Clinical Profile, Literature Review and Sixteen Year Experience from a Tertiary Care Teaching Institute. Indian J Pediatr 80, 1015–1020 (2013). https://doi.org/10.1007/s12098-013-1000-4

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  • DOI: https://doi.org/10.1007/s12098-013-1000-4

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