Rasmussen encephalitis: long-term outcome after surgery
Rasmussen encephalitis: long-term outcome after surgery
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DOI：
10.1007/s00381-008-0795-1
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发表时间：
2009-05-01
影响因子：
1.4
通讯作者：
Sakamoto, Americo C.
中科院分区：
文献类型：
--
作者：
Terra-Bustamante, Vera C.;Machado, Helio R.;Sakamoto, Americo C.
Rasmussen脑炎（RE）的特征是顽固性癫痫、进行性轻偏瘫和单侧大脑半球萎缩。症状进展为严重的神经功能障碍通常发生在几个月至几年内。RE的原因是未知的，虽然自身免疫过程的证据已在文献中广泛描述。抗癫痫药物通常不能有效控制癫痫发作或脑萎缩;尽管有数据支持早期免疫抑制和免疫调节干预的有益作用，但对于晚期RE患者的顽固性癫痫发作，半球分离形式的癫痫手术被认为是治疗的选择。这项工作描述了RE患者的临床和电图分析，以及手术后的演变。这项工作包括1995年1月至2008年1月由RibeirA o磅Preto Epilepsy Surgery Program（CIREP）评估的所有RE患者，采用诸如性别、癫痫发作年龄、癫痫发作症状学、癫痫发作频率、发作间期和发作期脑电图（EEG）结果等变量。手术时的年龄、癫痫持续时间、手术并发症、随访持续时间、解剖病理学发现、术后癫痫发作、语言和认知结果以及术后抗癫痫药物治疗。癫痫发作的平均年龄为4.4 ± 2.0岁。在癫痫发作年龄（p = 0.79）、手术年龄（p = 0.24）、癫痫持续时间（0.06）和随访（p = 0.40）方面，缓慢和快速进展患者之间无差异。存在双侧EEG异常或不存在棘波与术后癫痫发作结局之间无相关性（p = 0.06）。12例患者（48%）尝试了免疫调节治疗。23名患者接受了手术。平均随访63.3个月。11例患者癫痫发作完全控制。12例患者持续癫痫发作，包括轻度面部抽搐（6例患者），偶尔半全身强直阵挛性癫痫发作（3例患者）和频繁强直阵挛性癫痫发作（3例患者）。术后分别有15例和12例患者出现精神和语言障碍。8例患者出现术后认知功能下降，而只有2例患者有认知功能改善。12例语言障碍患者手术前后语言功能比较，66.7%的语言障碍患者术后无改善。患者被分为两组：轻偏瘫和持续性部分性癫痫的快速进展和缓慢进展。这些基团可以代表不同的RE底物。14例患者癫痫控制满意，3例患者对手术有部分反应，5例患者维持术前状态。所有左侧受累的患者均出现一定程度的语言障碍，66.6%的患者术后无改善。认知评估显示，大多数患者术后没有任何显著改善，38.1%的患者术后认知功能恶化。
Rasmussen encephalitis (RE) is characterized by intractable epilepsy, progressive hemiparesis, and unilateral hemispheric atrophy. The progression of the symptoms to significant neurological impairment usually occurs within months to a few years. RE causes are unknown, although evidence of an autoimmune process has been extensively described in the literature. Antiepileptic drugs are usually not effective to control seizures or cerebral atrophy; despite data supporting a beneficial effect of early immunosuppressive and immunomodulatory interventions, for intractable seizures in RE patients with advanced disease, epilepsy surgery in the form of hemispheric disconnection has been considered the treatment of choice. This work describes the clinical and electrographic analyses, as well as the post-operative evolution of patients with RE.This work includes all the patients with RE evaluated from January 1995 to January 2008 by the RibeirA o pound Preto Epilepsy Surgery Program (CIREP), taking variables such as gender; age at epilepsy onset; seizure semiology; seizure frequency; interictal and ictal electroencephalographic (EEG) findings; age at surgery, when done; duration of epilepsy; surgery complications; follow-up duration; anatomo-pathological findings; post-surgery seizure; language and cognitive outcome; and anti-epileptic drug treatment after surgery into account.Twenty-five patients were evaluated; thirteen were female. Mean age of epilepsy onset was 4.4 +/- 2.0 years. There were no differences between patients with slow and fast evolution with respect to age of epilepsy onset (p = 0.79), age at surgery (p = 0.24), duration of epilepsy (0.06), and follow-up (p = 0.40). There were no correlations between the presence of bilateral EEG abnormalities or the absence of spikes and post-operative seizure outcome (p = 0.06). Immunomodulatory therapy was tried in 12 patients (48%). Twenty-three patients underwent surgery. The mean follow-up was 63.3 months. Eleven patients had total seizure control. Twelve individuals persisted with seizures consisting of mild facial jerks (six patients), occasional hemigeneralized tonic-clonic seizures (three patients), and frequent tonic-clonic seizures (three patients). Mental and language impairment was observed in 15 and 12 patients, after surgery, respectively. Eight patients presented post-operative cognitive decline, while only two patients had cognitive improvement. Comparing pre- and post-operative language deficits, 66.7% of the 12 patients with language disturbance did not improve after surgery.This retrospective study reported the clinical and electrographic analysis, as well as the evolution of 23 patients with RE. Patients were divided into two groups: fast evolution and slow evolution to hemiparesis and epilepsia partialis continua. These groups may represent different RE substrates. Fourteen patients achieved satisfactory seizure control, three patients had partial response to surgery, and five patients had maintenance of the pre-operative condition. All patients with left-side involvement presented with some language disturbance, which did not improve after surgery in 66.6% of patients. Cognitive evaluation showed that the majority of the patients did not have any significant improvement, and 38.1% had cognitive deterioration after surgery.