Moyamoya is an angiogenic disorder and a rare cause of stroke. It is a progressive narrowing of cerebral arteries at the base of the brain involving the intracerebral portion of the internal carotid arteries, where it leads to development of collateral arteries, causing a "cigarette smoke" aspect. Ischemic events are more frequent in pediatric pathology, where hemorrhagic manifestations account for only 10%. The prognosis is severe, even worse than when onset of symptoms is earlier. We present the case of an 8-year-old child, who had presented recurrent hemiparesis since the age of 3 years; the brain CT scan and magnetic resonance imaging objectified injury sequelae. The MRA revealed stenosis of the internal carotid artery consistent with Moyamoya. In conclusion, the diagnosis of Moyamoya disease is now accessible to modern brain imaging techniques (MRI and MRA), easily achievable in children. The natural course of the disease involves the neurological and vital prognosis in young children, due to repeated ischemic attacks. Their identification would facilitate early diagnosis in order to treat neurosurgically, leading to a better neurological and cognitive prognosis.
Copyright © 2012. Published by Elsevier SAS.