Pediatric Moya Moya Disease: Clinical and Radio-Angiographic characteristics- A study from a Tertiary care center in Bangladesh

Background: Moya moya disease (MMD) is a cerebrovascular arteriopathy of unknown origin characterized by progressive stenosis followed by occlusion of the cerebral arteries. There is compensatory collaterals to perfuse the brain. There is pathogonomic angiographic appearance of puff of smoke in MMD. Very limited study has been done on MMD in pediatric population particularly in Bangladesh. This study thus has been done to highlight the clinical and neuroimaging features particularly angiographic features of MMD.

Methodology: We consecutively enrolled 40 children who have been diagnosed with MMD. This study has been done in a tertiary care hospital in Dhaka. Detail history regarding stroke, physical examination and MRI and MRA of brain was done in all patients. In selected cases, EEG and digital subtraction angiography was done. Results: We studied 40 patients, among them 23 had 1st attack and 8 had recurrent attacks of stroke. The common clinical presentation were hemiparesis, seizure and speech disorder. Fifteen (15) patients had bilateral involvement in the MRA. Most of the patients were in Suzuki Grading II and III. Conclusion: In children with stroke MMD are important etiology and often diagnosed late. This study will highlight the clinical and angiographic pattern of MMD in Bangladesh.
Keywords: Moya Moya Disease, Radio-Angiographic criteria