Published online May 6, 2023. doi: 10.12998/wjcc.v11.i13.3076
Peer-review started: December 19, 2022
First decision: February 17, 2023
Revised: February 23, 2023
Accepted: March 27, 2023
Article in press: March 27, 2023
Published online: May 6, 2023
Moyamoya disease (MMD) is a rare cause of acute stroke and transient ischemic attacks in children. We described clinical, diagnostic features and follow-ups of a young child with acute stroke.
We report a 4-year-old girl with left hemiparesis after an acute ischemic stroke. Her history was also significant for repeated left or right focal motor seizures, generalized tonic-clonic convulsions and transient ischemic attacks. Her magnetic resonance imaging and computed tomography (CT) of the brain and magnetic resonance angiography, CT angiography and venography on the cerebral vessels revealed evidence of bilateral fronto-parietal ischemic infarctions, occlusion of the right and left internal carotid arteries started at its bifurcation and non-visualization of right and left anterior and middle cerebral arteries. There was evidence of progression in angiography manifested as development of collaterals from the basal perforating vessels, increase in the extent of large intracranial arterial stenosis/occlusion and extensive collateral circulation with predominance from the posterior circulation. Physical and neurological evaluation and comprehensive laboratory investigations excluded an obvious comorbid disease or risk factor for the child’s condition. The diagnosis of MMD was highly suggested as a cause of the child’s steno-occlusive condition. She was treated symptomatically with levetiracetam, an antiepileptic medication. Aspirin was prescribed for secondary prevention. Her clinical manifestations were improved during the three years of follow-up. Revascularization surgery was postponed.
Up to our knowledge, this is the first report for MMD in a child in our country. The clinical improvement and the stabilization of the child’s condition over the 3 years of follow-up could be attributed to the rapid and extensive recruitment of collaterals and absence of risk factors or comorbidities. Revascularization surgery is highly recommended.
Core Tip: Stroke in children is a significant cause of long-lasting morbidity. The advances in neuroimaging and laboratory investigations serve an important role in proper evaluation of stroke in children and identification of its potential etiologies, risk factors and outcomes. Moyamoya disease (MMD) is a rare progressive non-inflammatory steno-occlusive arteriopathy of the large cerebral blood vessels. It is a rare cause of ischemic stroke and recurrent transient attacks in children. The disease is very under-recognized in different areas of the world except East Asia, predominantly Japan. MMD can be sporadic or familial. The Japanese term “moyamoya” refers to the puff of smoke morphology of the dilated basal collateral vessels within the brain tissue seen on cerebral angiography. Compared to other arteriopathies, MMD is unique as its treatment solely relies on surgical revascularization. Therefore, increasing reporting and evaluation of cases with MMD from different ethnicities may help in better understanding of its causes and proper management.