A SUCCESSFULLY TREATED CASE OF MOYA MOYA DISEASE

Moyamoya disease is a rare cerebrovascular disease characterized by progressive occlusive cerebral arteritis affecting the distal internal carotid arteries near the circle of Willis. A collateral circulation develops around the blocked vessels which appear as a "puff of smoke". Incidence is variable depending on the geographical location. The clinical features of Moyamoya disease are recurrent transient ischemic attacks (TIAs), strokes, sensorimotor paralysis, convulsions and/or migraine-like headaches. Magnetic resonance imaging (MRI) and MR Angiogram (MRA) should be performed for the diagnosis and follow-up of Moyamoya disease. Neurosurgical proceures like Encephalo myo synangiosis (EMS), multiple burr holes and some direct procedures are useful to reestablish new circulation after a few weeks. The long term outlook for patients with treated Moyamoya seems to be good. Once major stroke or bleeding take place, even with treatment, the patient may be left with permanent loss of function. So it is very important to treat this condition promptly. Herewith, we are reporting a 15 month old child with history of right focal adversive seizures, 3-4 events a month followed by post ictal drowsiness. Imaging studies revealed a vascular insult with infarct in the left fronto parietal lobes as well as multiple infarcts in other regions of the brain. MR Angio Brain revealed Moya moya disease. Child was successfully managed by prompt referral to Neurosurgical Centre where he underwent EMS and he is under their follow up.