Clinical and radio-angiographic features of paediatric moyamoya disease in Bangladesh

Abstract

Background: Moyamoya disease is a cerebrovascular arteriopathy of unknown origin characterized by progressive stenosis followed by occlusion of the cerebral arteries. Studies on moyamoya disease, especially in children in Bangladesh, are rare. We aimed to determine the clinical and neuroimaging features of moyamoya disease, particularly angiographic features.

Methods: Forty children diagnosed with moyamoya disease were consecutively recruited from Bangabandhu Sheikh Mujib Medical University Hospital, Dhaka, Bangladesh. Each patient underwent a medical history and physical examination focusing on stroke, magnetic resonance imaging, and magnetic resonance angiography scans of the brain. In some instances, electroencephalogram and digital subtraction angiography were also performed.

Results: Of the 40 patients, 22 experienced their first-ever stroke (median age, 84 months), and 18 had recurrent strokes (median age, 90 months). Common symptoms included hemiparesis, headache, seizure, and speech disorder. The commonly affected vessels were the internal carotid and middle cerebral arteries. Cortical involvement was found in 82.5% of cases. Bilateral involvement was observed in 37.5% of the patients, most of whom were in the Suzuki stage III.

Conclusion: Hemiparesis, headache, seizure, and speech disorder were the common manifestations. Most patients reported late (Suzuki stages III and IV), indicating an advanced stage.  Early detection is necessary, considering the severity of the disease and its inherent tendency for recurrence.