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癫痫发作作为III型斯特奇-韦伯综合征的首发症状:两例报告。

Epileptic seizures as an initial symptom for Sturge‑Weber syndrome type III: A report of two cases.

作者信息

Wang Yaping, Geng Guifu, Hu Wandong, Zhang Huan, Liu Yong, Gao Zaifen, Zhang Hongwei, Shi Jianguo

机构信息

Epilepsy Center, Jinan Children's Hospital (Children's Hospital Affiliated to Shandong University), Jinan, Shandong 250000, P.R. China.

出版信息

Exp Ther Med. 2024 May 24;28(1):299. doi: 10.3892/etm.2024.12588. eCollection 2024 Jul.

Abstract

Sturge-Weber syndrome (SWS) type III, a rare neurocutaneous disorder, presents diagnostic challenges due to its variable clinical manifestations. The present study focuses on enhancing the understanding of this syndrome by conducting a detailed analysis of two pediatric cases and providing a comprehensive review of the existing literature. The cases, managed at the Children's Hospital Affiliated to Shandong University (Jinan, China), highlight the diverse clinical presentations and successful management strategies for SWS type III. In the first case, a 4-year-old male patient exhibited paroxysmal hemiplegia, epileptic seizures and cerebral angiographic findings indicative of left pia mater and venous malformation. The second case involved a 2.5-year-old male patient presenting with recurrent seizures and angiographic findings on the right side. Both cases underscore the importance of considering epileptic seizures, acquired and transient hemiplegia and cognitive impairments in the diagnosis of SWS type III. The present study provides insights into the effective use of both pharmacological and surgical interventions, drawing from the positive outcomes observed in these cases. The findings emphasize the need for heightened awareness and a meticulous approach in diagnosing and treating SWS type III, contributing to the better management and prognosis of this condition.

摘要

III型斯特奇-韦伯综合征(SWS)是一种罕见的神经皮肤疾病,因其临床表现多样而带来诊断挑战。本研究通过对两例儿科病例进行详细分析并对现有文献进行全面综述,着重加深对该综合征的理解。这两例病例在山东大学附属儿童医院(中国济南)接受治疗,突出了III型SWS的多样临床表现及成功的治疗策略。在第一例病例中,一名4岁男性患者表现为阵发性偏瘫、癫痫发作以及脑部血管造影结果显示左侧软脑膜和静脉畸形。第二例病例涉及一名2.5岁男性患者,表现为反复癫痫发作且右侧有血管造影结果。两例病例均强调了在III型SWS诊断中考虑癫痫发作、后天性和短暂性偏瘫以及认知障碍的重要性。本研究从这些病例中观察到的积极结果出发,深入探讨了药物和手术干预的有效应用。研究结果强调了在诊断和治疗III型SWS时提高认识和采取细致方法的必要性,有助于改善该疾病的管理和预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/246f/11168029/fbd85d5ec38c/etm-28-01-12588-g00.jpg

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