Faciobrachial Dystonic Seizures Precede Lgi1 Antibody Limbic Encephalitis
Faciobrachial Dystonic Seizures Precede Lgi1 Antibody Limbic Encephalitis
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DOI：
10.1002/ana.22307
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发表时间：
2011-05-01
影响因子：
11.2
通讯作者：
Vincent, Angela
中科院分区：
文献类型：
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作者：
Irani, Sarosh R.;Michell, Andrew W.;Vincent, Angela
目的：描述一个独特的癫痫发作的症状，密切相关的电压门控钾通道（VGKC）-复合物/Lgi 1抗体，通常发生在边缘系统脑炎（LE）的发病之前。方法：29例患者被确定的作者（n = 15）或参考临床医生（n = 14）。临床特征和血清钠，脑磁共振成像（MRI），正电子发射断层扫描/单光子发射计算机断层扫描，和VGKC-复合抗体的时间进展进行了study.Results：视频和静止图像显示了一个独特的成人发病，频繁，短暂的张力障碍性癫痫症状，主要影响手臂和同侧脸。我们称之为面臂肌张力障碍性癫痫发作（FBDS）。所有在患病期间检测的患者都有VGKC复合物的抗体; 89%的特异性抗原靶点是Lgi 1。尽管3例患者从未发生LE，但其余26例患者中的20例（77%）在发生LE特征性失忆和意识模糊之前发生了FBDS。在单独FBDS的前驱症状期间，患者的钠和脑MRI正常，但脑电图显示7例患者（24%）有发作性癫痫样活动。在LE发展后，患者经常发展其他癫痫发作症状，包括典型的内侧颞叶癫痫发作。在此阶段，检查通常显示低钠血症和MRI海马高T2信号;脑功能成像显示5/8例基底节受累的证据。抗癫痫药物（AEDs）通常无效，41%的药物与皮肤反应有关，通常是严重的。相比之下，免疫疗法产生了明确的，往往是戏剧性的，减少FBDS frequency.Interpretation：识别FBDS应提示VGKC复合物/Lgi 1抗体的测试。抗癫痫药物通常会产生不良反应;免疫治疗可能会预防LE的发展，并可能导致脑萎缩和认知障碍。神经网络2011; 69：892-900
Objective: To describe a distinctive seizure semiology that closely associates with voltage-gated potassium channel (VGKC)-complex/Lgi1 antibodies and commonly precedes the onset of limbic encephalitis (LE).Methods: Twenty-nine patients were identified by the authors (n = 15) or referring clinicians (n 14). The temporal progression of clinical features and serum sodium, brain magnetic resonance imaging (MRI), positron emission tomography/single photon emission computed tomography, and VGKC-complex antibodies was studied.Results: Videos and still images showed a distinctive adult-onset, frequent, brief dystonic seizure semiology that predominantly affected the arm and ipsilateral face. We have termed these faciobrachial dystonic seizures (FBDS). All patients tested during their illness had antibodies to VGKC complexes; the specific antigenic target was Lgi1 in 89%. Whereas 3 patients never developed LE, 20 of the remaining 26 (77%) experienced FBDS prior to the development of the amnesia and confusion that characterize LE. During the prodrome of FBDS alone, patients had normal sodium and brain MRIs, but electroencephalography demonstrated ictal epileptiform activity in 7 patients (24%). Following development of LE, the patients often developed other seizure semiologies, including typical mesial temporal lobe seizures. At this stage, investigations commonly showed hyponatremia and MRI hippocampal high T2 signal; functional brain imaging showed evidence of basal ganglia involvement in 5/8. Antiepileptic drugs (AEDs) were generally ineffective and in 41% were associated with cutaneous reactions that were often severe. By contrast, immunotherapies produced a clear, and often dramatic, reduction in FBDS frequency.Interpretation: Recognition of FBDS should prompt testing for VGKC-complex/Lgi1 antibodies. AEDs often produce adverse effects; treatment with immunotherapies may prevent the development of LE with its potential for cerebral atrophy and cognitive impairment. ANN NEUROL 2011; 69: 892-900