Episodic bradycardia as neurocardiac prodrome to voltage-gated potassium channel complex/leucine-rich, glioma inactivated 1 antibody encephalitis.
Episodic bradycardia as neurocardiac prodrome to voltage-gated potassium channel complex/leucine-rich, glioma inactivated 1 antibody encephalitis.
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DOI：
10.1001/jamaneurol.2014.1234
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发表时间：
2014-10
期刊：
影响因子：
29
通讯作者：
Gelfand, Jeffrey M.
中科院分区：
文献类型：
--
作者：
Naasan, Georges;Irani, Sarosh R.;Bettcher, Brianne M.;Geschwind, Michael D.;Gelfand, Jeffrey M.
电压门控钾通道复合物抗体（VGKCc-Ab）脑炎是一种免疫治疗应答综合征，通常与靶向富含亮氨酸的胶质瘤失活1（LGI 1）蛋白的致病抗体相关。虽然它在整个大脑中表达，但LGI 1在心脏组织中表达尚不清楚。我们描述了一种新的VGKCc-Ab/LGI 1脑炎的神经心脏前驱症状。在加州大学弗朗西斯科分校自身免疫性脑炎诊所和快速痴呆研究项目中评价的14例VGKCc/LGI 1-Ab脑炎患者中，3例患者（2例男性和1例女性;年龄53岁、55岁和64岁）在脑病发作前约2个月出现阵发性心动过缓，严重程度足以导致起搏器植入。在随后的脑病时检测血清LGI 1-Ab结果为阳性。所有3例患者均发生低钠血症;无面臂肌张力障碍性癫痫发作或恶性肿瘤。脑磁共振成像异常2例。随访1.7 - 7年后，所有患者均未出现进一步的症状性心律失常。发作性心动过缓是VGKCc/LGI 1-Ab脑炎的一种独特的神经心脏前驱症状。神经解剖定位最可能涉及岛叶和颞叶受累，调节心脏自主神经功能的皮质区域。需要进一步的研究来确定识别这种神经心脏前驱症状和早期免疫抑制是否可以预防脑病的发展。
Voltage-gated potassium channel complex antibody (VGKCc-Ab) encephalitis is an immunotherapy-responsive syndrome usually associated with causative antibodies that target the leucine-rich, glioma inactivated 1 (LGI1) protein. Although it is expressed throughout the brain, LGI1 is not known to be expressed in cardiac tissue. We describe a novel neurocardiac prodrome of VGKCc-Ab/LGI1-encephalitis. Among 14 patients with VGKCc/LGI1-Ab encephalitis evaluated in the University of California, San Francisco Autoimmune Encephalitis Clinic and Rapid Dementia Research Program, 3 patients (2 men and 1 woman; aged 53, 55, and 64 years) exhibited episodic bradycardia that preceded the onset of encephalopathy by approximately 2 months and was severe enough to lead to pacemaker implantation. Serum LGI1-Ab results were positive when tested at the time of the subsequent encephalopathy. All 3 patients developed hyponatremia; none had faciobrachial dystonic seizures or malignancy. Brain magnetic resonance imaging was abnormal in 2 cases. None of the patients experienced further symptomatic bradyarrythmias after 1.7 to 7 years of follow-up. Episodic bradycardia is a distinctive neurocardiac prodrome of VGKCc/LGI1-Ab encephalitis. The neuroanatomical localization most likely relates to insular and temporal lobe involvement, cortical regions that modulate cardiac autonomic function. Further study is needed to determine if recognition of this neurocardiac prodrome and earlier institution of immunosuppression can prevent the development of encephalopathy.
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影响因子：
11.2
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