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自身免疫性脑炎相关癫痫发作的临床电特征。

Electroclinical features of seizures associated with autoimmune encephalitis.

机构信息

Epilepsy Center, Cleveland Clinic Foundation, Cleveland, OH, USA.

Epilepsy Center, Cleveland Clinic Foundation, Cleveland, OH, USA.

出版信息

Seizure. 2018 Aug;60:198-204. doi: 10.1016/j.seizure.2018.06.021. Epub 2018 Jun 28.

Abstract

PURPOSE

We sought to characterize the electroclinical features of seizures associated with autoimmune encephalitis and their relevance to outcome.

METHODS

19 patients with seizures and autoimmune encephalitis were identified from a database of 100 patients (2008-2017) with autoimmune neurological disorders. Clinical and electroclinical characteristics were collected. Persistent seizures at last follow-up were then correlated with electroclinical features.

RESULTS

Status epilepticus (53%) and early intractability to AEDs (median time to second AED 9.5 days) marked the onset of refractory seizures (median number of AEDs 3). Seizure semiology (abdominal (16%), psychic (42%), olfactory (6%) auras), interictal temporal epileptiform discharges (42%), and ictal onset in the temporal region (63%) mirrored radiologic involvement of the medial temporal regions (on MRI in 74% and/or FDG-PET in 75%). In addition, multimodal auras, with somatosensory (26%), autonomic (26%), gustatory (11%), and visual (16%), features were seen in 82% of patients with focal aware seizures, invoking broader involvement of the perisylvian regions. A change in seizure semiology and EEG findings was often seen. Electroclinical features were similar regardless of antibody type, with the exception of the association of faciobrachial dystonic seizures with LGI1 antibodies. Eight patients had medically intractable seizures at last follow-up and were more likely than patients with seizure remission to have generalized tonic-clonic seizures and temporal lobe involvement on the basis of semiological features, interictal EEG and MRI changes.

CONCLUSIONS

Seizures associated with autoimmune encephalitis exhibit common electroclinical features which show dynamic evolution over time. We propose a role for the temporo-perisylvian regions in their generation.

摘要

目的

我们旨在描述与自身免疫性脑炎相关的癫痫发作的电临床特征及其与结局的相关性。

方法

从 100 例(2008-2017 年)自身免疫性神经疾病患者的数据库中确定了 19 例癫痫发作和自身免疫性脑炎患者。收集临床和电临床特征。然后将最后一次随访时的持续性癫痫发作与电临床特征相关联。

结果

癫痫持续状态(53%)和早期对抗癫痫药物的耐药性(使用第二种抗癫痫药物的中位数时间为 9.5 天)标志着难治性癫痫发作的开始(中位数使用抗癫痫药物的数量为 3)。癫痫发作的半影(腹部(16%)、精神(42%)、嗅觉(6%)先兆)、发作间期颞叶癫痫样放电(42%)和颞区发作起始(63%)反映了内侧颞区的影像学受累(74%在 MRI 上,75%在 FDG-PET 上)。此外,82%的局灶性意识清醒的癫痫发作患者出现了多模态先兆,伴有躯体感觉(26%)、自主神经(26%)、味觉(11%)和视觉(16%)特征,提示累及了更广泛的岛叶区域。经常可以看到癫痫发作半影和脑电图发现的变化。电临床特征与抗体类型无关,除了面臂肌张力障碍性癫痫发作与 LGI1 抗体有关外。8 例患者在最后一次随访时癫痫发作仍难以控制,与癫痫缓解的患者相比,他们更有可能出现全面强直阵挛性发作和基于半影特征、发作间期脑电图和 MRI 变化的颞叶受累。

结论

与自身免疫性脑炎相关的癫痫发作表现出常见的电临床特征,这些特征随着时间的推移呈现动态演变。我们提出了颞-岛叶区域在其发生中的作用。

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