Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis.
Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis.
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DOI：
10.1097/wco.0000000000000449
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发表时间：
2017-06
影响因子：
4.8
通讯作者：
Dalmau J
中科院分区：
文献类型：
--
作者：
Spatola M;Dalmau J
评估与神经元细胞表面（AE）或髓鞘相关抗原抗体相关的脑炎中癫痫发作的表现和风险，并回顾几种慢性癫痫疾病，包括拉斯穆森脑炎（RE）、发热诱导的难治性癫痫综合征（FIRES）和新发难治性癫痫持续状态（NORSE）。癫痫发作是AE的常见表现。一些AE可能与特征性特征相关：面臂张力障碍性癫痫发作（抗LGI 1脑炎）、EEG极端δ刷波（抗NMDAR）或多灶性FLAIR-MRI异常（抗GABAAR）。在抗LGI 1脑炎中，皮质、边缘和基底神经节功能障碍导致不同类型的癫痫发作。AE或髓鞘抗体相关综合征通常是免疫治疗反应性的，并且似乎具有慢性癫痫的低风险。相反，与GAD 65抗体（一种细胞内抗原）相关的癫痫发作患者经常发生癫痫，对治疗（包括手术）的反应不佳。RE或FIRES可能与意义不明的自身抗体一起发生，很少对免疫治疗有反应。一项对NORSE患者的研究表明，30%的患者患有慢性癫痫。虽然癫痫发作在所有类型的AE中都很常见，但慢性癫痫的风险取决于抗原：如果位于细胞表面，则风险较低，如果位于细胞内，则风险较高。对于其他疾病（RE、FIRES、NORSE），预后仍然很差。
To assess the seizure manifestations and risk of epilepsy in encephalitis associated to antibodies against neuronal cell-surface (AE) or myelin-associated antigens, and to review several chronic epileptic disorders including, Rasmussen’s encephalitis (RE), fever-induced refractory epileptic syndromes (FIRES), and new-onset refractory status epilepticus (NORSE). Seizures are a frequent manifestation of AE. Some AE may associate with characteristic features: faciobrachial dystonic seizures (anti-LGI1 encephalitis), EEG extreme delta brush (anti-NMDAR), or multifocal FLAIR-MRI abnormalities (anti-GABAAR). In anti-LGI1 encephalitis, cortical, limbic, and basal ganglia dysfunction results in different types of seizures. AE or myelin-antibody associated syndromes are often immunotherapy-responsive and appear to have a low risk for chronic epilepsy. In contrast patients with seizures related to GAD65-antibodies (an intracellular antigen) frequently develop epilepsy and have suboptimal response to treatment (including surgery). RE or FIRES may occur with autoantibodies of unclear significance and rarely respond to immunotherapy. A study of patients with NORSE showed that 30% developed chronic epilepsy. Although seizures are frequent in all types of AE, the risk for chronic epilepsy is dependent on the antigen: lower if located on the cell-surface, and higher if intracellular. For other disorders (RE, FIRES, NORSE) the prognosis remains poor.