Functional hemispherotomy in Rasmussen syndrome in the absence of classic MRI findings

Yasunori Nagahama , Charuta Joshi , Brian Dlouhy , Angela Y. Wu , Taylor J. Abel , Gary Baumbach , Hiroto Kawasaki
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引用次数: 5

Abstract

A 7-year-old previously healthy girl presented with a left-sided focal seizure without impaired consciousness and subsequently developed epilepsia partialis continua. Initial MRI was normal, and the subsequent images only showed a focal T2/FLAIR hyperintense area without cortical atrophy. She was diagnosed with Rasmussen syndrome by pathology and promptly treated with functional hemispherotomy. Rasmussen syndrome is a rare progressive neurological disorder, the only definitive cure for which is hemispheric disconnection. The disease presents a management dilemma, especially early in disease course without characteristic neuroimaging features. A high index of suspicion, multidisciplinary approach, and clear timely communication with the family are critical.

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在没有典型MRI表现的情况下,Rasmussen综合征的功能性半球切除术
一个7岁的健康女孩表现为左侧局灶性癫痫,没有意识受损,随后发展为部分连续性癫痫。最初的MRI正常,随后的图像仅显示局灶性T2/FLAIR高信号区,无皮质萎缩。病理诊断为拉斯穆森综合征,并及时行功能性半球切开术治疗。拉斯穆森综合征是一种罕见的进行性神经系统疾病,唯一有效的治疗方法是大脑半球断开。该疾病呈现出一个管理困境,特别是在病程早期没有特征性的神经影像学特征。高度的怀疑、多学科的方法和与家人及时明确的沟通是至关重要的。
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