Hamed Sherifa Ahmed, Yousef Hosam Abozaid
Department of Neurology and Psychiatry, Assiut University, Faculty of Medicine, Assiut 71516, Assiut, Egypt.
Department of Radiology, Assiut University, Faculty of Medicine, Assiut 71516, Assiut, Egypt.
World J Clin Cases. 2023 May 6;11(13):3076-3085. doi: 10.12998/wjcc.v11.i13.3076.
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.
烟雾病（MMD）是儿童急性卒中及短暂性脑缺血发作的罕见病因。我们描述了一名急性卒中幼儿的临床、诊断特征及随访情况。
我们报告一名4岁急性缺血性卒中后出现左侧偏瘫的女孩。她既往有反复的左侧或右侧局灶性运动性癫痫发作、全身强直阵挛性惊厥及短暂性脑缺血发作病史。她的脑部磁共振成像（MRI）、计算机断层扫描（CT）以及脑血管磁共振血管造影（MRA）、CT血管造影（CTA）和静脉造影显示双侧额顶叶缺血性梗死，左右颈内动脉自分叉处开始闭塞，左右大脑前动脉及大脑中动脉不显影。血管造影显示有病情进展，表现为基底穿支血管侧支形成、颅内大动脉狭窄/闭塞范围扩大以及以后循环为主的广泛侧支循环。体格检查、神经学评估及全面实验室检查排除了该患儿病情的明显合并症或危险因素。高度怀疑烟雾病是导致该患儿狭窄闭塞性病变的病因。给予抗癫痫药物左乙拉西坦对症治疗。给予阿司匹林进行二级预防。在三年随访期间其临床表现有所改善。血管重建手术推迟进行。
据我们所知，这是我国首例儿童烟雾病报告。患儿在三年随访期间临床症状改善且病情稳定，这可能归因于侧支循环迅速广泛形成以及无危险因素或合并症。强烈推荐进行血管重建手术。
