Atypical presentation in Rasmussen encephalitis: delayed late-onset periodic epileptic spasms
Atypical presentation in Rasmussen encephalitis: delayed late-onset periodic epileptic spasms
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DOI：
10.1684/epd.2011.0455
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发表时间：
2011-09-01
影响因子：
2.3
通讯作者：
Yacubian, Elza Marcia T.
中科院分区：
文献类型：
--
作者：
Ferrari, Taissa P. F.;Hamad, Ana P. A.;Yacubian, Elza Marcia T.
一个五岁半的女孩在两岁半的时候开始经历进行性左侧轻偏瘫。在5岁零4个月时，她开始出现不对称周期性癫痫痉挛，无心律失常。发作期脑电图表现为周期性、恒定性和刻板性复合波。连续脑成像显示右半球进行性萎缩，MRI上T2信号增加。她接受了右半球切开术，组织学检查显示炎症迹象和局灶性皮质发育不良（FCD）的特征。她已经16个月没有吸毒了。该病例在以下方面是独特的：典型的拉斯穆森脑炎的特征是进行性单侧脑受累，无癫痫发作，癫痫发作前延迟近3年;非典型癫痫发作类型表现为周期性癫痫痉挛，存在与炎症变化相关的FCD。[发布视频序列]
A five-and-a-half-year-old girl started experiencing progressive left hemiparesis at age two and a half years. At age five years and four months she started presenting clusters of asymmetric periodic epileptic spasms with no hypsarrhythmia. The ictal EEG showed periodic, constant and stereotyped complexes. Serial brain imaging revealed progressive atrophy of the right hemisphere with increased T2 signal on MRI. She underwent a right hemispherotomy, and histological examination showed signs of inflammation and features of focal cortical dysplasia (FCD). She has been seizure-free for 16 months. This case is unique in the following aspects: the presence of typical Rasmussen encephalitis features of progressive unilateral brain involvement without seizures, a delay of almost three years prior to seizure onset; an atypical seizure type presentation with periodic epileptic spasms and the presence of FCD associated with inflammatory changes. [Published with video sequences]