Case Report
Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. May 6, 2023; 11(13): 3076-3085
Published online May 6, 2023. doi: 10.12998/wjcc.v11.i13.3076
Idiopathic steno-occlusive disease with bilateral internal carotid artery occlusion: A Case Report
Sherifa Ahmed Hamed, Hosam Abozaid Yousef
Sherifa Ahmed Hamed, Department of Neurology and Psychiatry, Assiut University, Faculty of Medicine, Assiut 71516, Assiut, Egypt
Hosam Abozaid Yousef, Department of Radiology, Assiut University, Faculty of Medicine, Assiut 71516, Assiut, Egypt
Author contributions: Hamed SA performed the clinical follow-ups of the patient, wrote the manuscript and had the final responsibility to submit the manuscript for publication; Yousef HA performed the neuroimaging, verified the underlying data and interpretation of the results and final approval of the manuscript.
Informed consent statement: The consent form to treat the child and to publish the child’s clinical, laboratory and imaging date and treatment has been signed by the child’s father.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read CARE Checklist (2016), and the manuscript was prepared and revised according to CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Sherifa Ahmed Hamed, MD, Professor, Department of Neurology and Psychiatry, Assiut University, Faculty of Medicine, Assiut University Hospital Street, No. 1, Assiut 71516, Assiut, Egypt. hamedsherifa@aun.edu.eg
Received: December 19, 2022
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
Processing time: 126 Days and 11.7 Hours
Abstract
BACKGROUND

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.

CASE SUMMARY

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.

CONCLUSION

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.

Keywords: Steno-occlusive disease; Moyamoya disease; Internal carotid artery; Collateral circulation; Neo-vascularization; Case report

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.