An unusual presentation of Moyamoya disease managed conservatively in a resource limited region: A case report

Moyamoya disease is a rare cerebrovascular disease of chronic and progressive nature, characterized by stenosis and occlusion of the Circle of Willis vessels and formation of compensatory collateral circulation. Here we have reported a case of a 10 year old male patient who first developed seizure, hemiparesis 1 year ago, followed by deteriorating hemiparesis, prolonged fever and blindness. Although seizure hemiparesis are common presentations of the disease, blindness and prolonged fever have been rarely reported. The diagnosis in this case was established by Magnetic Resonance Imaging (MRI) and Magnetic Resonance Angiogram (MRA), where vessel occlusion, encephalomalacia and compensatory circulation formation were detected. Due to financial hardships and limitations of the surgical settings in the hospital, conservative treatment was provided. In fact, there has been no report of performing revascularization surgery in a patient with Moyamoya disease in Bangladesh. This case highlights that progressive cortical blindness and posterior circulation involvement may occur in pediatric Moyamoya disease. In resource-limited settings, characteristic MRI and MRA findings can facilitate early diagnosis when Digital Subtraction Angiography is unavailable, although definitive management with revascularization surgery remains the preferred treatment.