Epilepsy surgery in adult-onset Rasmussen's encephalitis: case series and review of the literature
Epilepsy surgery in adult-onset Rasmussen's encephalitis: case series and review of the literature
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DOI：
10.1007/s10143-015-0623-5
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发表时间：
2015-07-01
影响因子：
2.8
通讯作者：
Di Gennaro, Giancarlo
中科院分区：
文献类型：
--
作者：
Casciato, Sara;Di Bonaventura, Carlo;Di Gennaro, Giancarlo
拉斯穆森脑炎（RE）是一种罕见的免疫介导的疾病，其特征是抗药性局灶性癫痫，进行性神经和认知缺陷与单侧半球萎缩。发病通常在儿童期报告，尽管已描述了成人病例（A-RE）。虽然儿童RE的手术策略是明确的，但对A-RE患者癫痫手术的有用性知之甚少。我们描述了5例接受癫痫手术的A-RE患者的临床特征、手术方法和结局，并回顾了关于手术A-RE病例的文献。我们回顾性研究了5例年龄在21-38岁（平均年龄22.8岁）的A-RE患者，他们在手术后随访了1 - 6年。人口统计学，电临床和神经影像学数据进行了系统的审查。5名受试者中有4名接受了侵入性EEG监测以确定致痫区。根据恩格尔的分类定义癫痫结局。手术包括额叶皮质切除术3例，颞叶切除术1例，颞叶加岛叶联合切除术1例，额基底皮质切除术1例。术后未观察到永久性神经功能缺损。在末次随访观察时，1例患者无癫痫，2例受试者发生罕见致残性癫痫发作，另1例患者癫痫发作中度减少，1例患者无临床改善。我们的经验，虽然仅限于少数情况下，表明切除手术在A-RE可能发挥作用的背景下，多学科的治疗方法，这种严重的条件。由于缺乏关于手术选择的具体数据，这一主题似乎值得进一步调查和更有针对性的研究。
Rasmussen's encephalitis (RE) is a rare immunemediated condition characterized by drug-resistant focal epilepsy, progressive neurological, and cognitive deficits associated to unilateral hemispheric atrophy. The onset is typically reported in childhood, although adult cases (A-RE) have been described. While surgical strategies in childhood RE are well defined, little is known about usefulness of epilepsy surgery in A-RE patients. We describe clinical features, surgical approach, and outcome of five A-RE patients who underwent epilepsy surgery, and we review the literature with regard to surgical A-RE cases. We retrospectively studied five A-RE patients aged 21-38 years (mean age 22.8 years) who were followed after surgery for a period ranging from to 1 to 6 years. Demographic, electroclinical, and neuroimaging data were systematically reviewed. Four out of five subjects underwent invasive EEG monitoring to define epileptogenic zone. Epilepsy outcome was defined according to Engel's classification. Surgery consisted of frontal corticectomy in three patients, temporal lobectomy in one, combined temporal lobectomy plus insular, and frontobasal corticectomy in the remaining case. No permanent neurological deficits were observed after surgery. At the last follow-up observation, one patient was seizure-free, two subjects experienced rare disabling seizures, another had moderate seizure reduction, and one had no clinical improvement. Our experience, although limited to few cases, suggests that resective surgery in A-RE may play a role in the context of multidisciplinary therapeutical approach of this severe condition. Since the lack of specific data about surgical options, this topic seems to deserve further investigations and more targeted studies.