Characteristics of clinical evolution,EEG and neuroimaging in Rasmussen’s syndrome

LIANG Jinping

doi:10.13406/j.cnki.cyxb.2007.07.023

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Characteristics of clinical evolution,EEG and neuroimaging in Rasmussen’s syndrome

更新时间：2024-01-17

- Characteristics of clinical evolution,EEG and neuroimaging in Rasmussen’s syndrome
- Journal of Chongqing Medical University Issue 7, Pages: 722-726(2007)
- 作者机构：
  
  1. 重庆医科大学儿童医院神经内科
  2. 重庆市第三人民医院放射科
- 作者简介：
- 基金信息：
- DOI：10.13406/j.cnki.cyxb.2007.07.023
  CLC： R742.1
- Published：2007，
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[1]梁锦平,冯成功,扬华.Rasmussen综合征的临床过程及脑电图和神经影像学特征[J].重庆医科大学学报,2007(07):722-726.

LIANG Jinping. Characteristics of clinical evolution,EEG and neuroimaging in Rasmussen’s syndrome. [J]. Journal of Chongqing Medical University (7):722-726(2007)
[1]梁锦平,冯成功,扬华.Rasmussen综合征的临床过程及脑电图和神经影像学特征[J].重庆医科大学学报,2007(07):722-726. DOI： 10.13406/j.cnki.cyxb.2007.07.023.

LIANG Jinping. Characteristics of clinical evolution,EEG and neuroimaging in Rasmussen’s syndrome. [J]. Journal of Chongqing Medical University (7):722-726(2007) DOI： 10.13406/j.cnki.cyxb.2007.07.023.

摘要

目的:探讨Rasmussen综合征的临床过程及脑电图和神经影像学特征。方法:借助EEG、CT和MRI技术

进行临床观察和药物治疗及复习文献资料。结果:本组2例患儿均经过1～2年的临床随访

进行了必要的辅助检查和正规的抗癫痫治疗

临床确诊。起病年龄分别为2岁2月和6岁8月;以反复局灶性癫痫发作、慢性进行性部分性癫痫持续状态和一侧优势的全面性癫痫发作为主

伴进行性偏瘫和认知功能障碍和语言障碍（优势半球受累）;EEG示持续性局灶慢活动异常伴后期棘慢波发放;MRI显示逐渐加重的局灶性高信号果影和脑萎缩;常规抗痫疗效差。结论:Rasmussen综合征是一种特殊类型的局灶性癫痫综合征

具有独特的临床表现以及脑电图和影像学特征

其病理生理和治疗措施具有研究价值。

Abstract

Objective:To identify the presence of clinical evolution

electroencephalographic and neuroimaging patterns of Ras- mussen’s syndrome（RS）.Methods:By means of EEG

MRI and observation of clinical evolution and drug therapy as well as reviewing the referential data.Results:The two children with Rasmussen’s syndrome were the age of onset between 26 months and 6 year 8 months

characterized by repetitive focal epileptic seizures or chronic progressive epilepsia partialis continua and secondarily generalized convulsive seizures dominant from one side followed by slowly progressive neurological deterioration in- cluding hemiparesis

cognitive impairment and dysphasia

where the dominant hemisphere is involved.Focal delta and theta or delta intermingled slowly with small spikes or sharp waves were steadily recorded in EEG;Radiological evidence of increased signal intensity on T

-weighted images and progressive

usually unilateral

cerebral atrophy are highly suggestive of RS;Conven- tional antiepileptic drug therapies are poorly responsive.Conclusion:Rasmussen’s syndrome is an unusual pattern of focal epileptic syndrome characterized by unique clinical evolution

electroencephalographic and neuroimaging changes

in which the pathophysiology and optimum treatment are worthy of being explored.

关键词

Rasmussen综合征临床表现脑电图神经影像学

Keywords

Rasmussen’s syndromeclinical manifestationEEGneuroimaging

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