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戴克-戴维多夫-马森综合征:后天性大脑半球萎缩的罕见病因。

Dyke-Davidoff-Masson Syndrome: A Rare Cause of Acquired Cerebral Hemiatrophy.

作者信息

Bhol Deepak, Chandrasekar Shyam, John Joseph, Satapathy Amit Kumar

机构信息

Department of Pediatrics, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India.

出版信息

Asian J Neurosurg. 2021 Sep 14;16(3):579-581. doi: 10.4103/ajns.AJNS_499_20. eCollection 2021 Jul-Sep.

Abstract

Dyke-Davidoff-Masson syndrome is a rare disease of childhood which is clinically characterized by hemiparesis, refractory seizures, facial asymmetry, and mental retardation. The classical radiological findings are cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses. Seizure refractory to medical management warrants surgical intervention with excellent outcome. Here, we are reporting two such cases who presented late and diagnosis was made on the basis of magnetic resonance imaging brain features. Both of our children responded to oral anticonvulsant and are on regular follow-up.

摘要

戴克-戴维多夫-马森综合征是一种儿童罕见病,临床特征为偏瘫、难治性癫痫、面部不对称和智力发育迟缓。典型的放射学表现为脑半侧萎缩、颅骨增厚和额窦过度气化。药物治疗无效的癫痫发作需要进行手术干预,效果良好。在此,我们报告两例出现较晚的此类病例,根据脑部磁共振成像特征做出诊断。我们的两个患儿对口服抗惊厥药物有反应,正在定期随访。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69c7/8477814/7354ffa22bf7/AJNS-16-579-g001.jpg

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