Chowdhry Mohit, Gajulapalli Shiva Prasad, Agrawal Soma
Department of Transfusion Medicine, Indraprastha Apollo Hospitals, New Delhi, India.
Department of Transfusion Medicine, Indraprastha Apollo Hospitals, New Delhi, India.
Transfus Apher Sci. 2020 Feb;59(1):102590. doi: 10.1016/j.transci.2019.07.003. Epub 2019 Jul 22.
Neurological syndromes associated with voltage-gated potassium channels (VGKC) affect the nerve and muscle physiology. Presence of antibodies to VGKC are associated with three main neurologic syndromes namely neuromyotonia (NMT), limbic encephalitis (LE) and Morvan's syndrome(MVS) LE is a variably treatable neurologic syndrome associated with high levels of antibodies to the voltage-gated potassium channel (VGKC) complex. These antibodies are directed against protein antigens that bind to the VGKC complex. These antigens are usually leucine-rich, glioma inactivated 1 (LGI1), and contactin associated protein-like 2 (CASPR2).
A 58-year-old female and with a known case of auto immune encephalitis (voltage gated potassium channel) and steroid induced diabetes mellitus presented with progressive worsening of vertigo, recurrent myoclonic jerks and post ictal confusion for last 7 days. She had memory impairment since last few months. She was on treatment with steroids which were gradually tapered off 11 months back. CSF was tested for presence of VGKC antibodies and the test was positive for LGI (leucine-rich glioma inactivated 1) antibody. Therapeutic plasma exchange (TPE) was scheduled every day for 6 consecutive days based upon the recommendations from the ASFA guidelines for the treatment of neurologic syndromes.
TPE done every day in patient diagnosed LE with VGKC antibodies had shown rapid improvement in controlling the symptoms.
与电压门控钾通道（VGKC）相关的神经综合征会影响神经和肌肉生理功能。存在针对VGKC的抗体与三种主要神经综合征相关，即神经性肌强直（NMT）、边缘叶脑炎（LE）和莫旺综合征（MVS）。LE是一种可通过多种方式治疗的神经综合征，与高水平的电压门控钾通道（VGKC）复合物抗体有关。这些抗体针对与VGKC复合物结合的蛋白质抗原。这些抗原通常是富含亮氨酸的胶质瘤失活1（LGI1）和接触蛋白相关蛋白样2（CASPR2）。
一名58岁女性，已知患有自身免疫性脑炎（电压门控钾通道型）和类固醇诱导的糖尿病，在过去7天中出现眩晕进行性加重、反复肌阵挛性抽搐和发作后意识模糊。她在过去几个月中存在记忆障碍。她曾接受类固醇治疗，11个月前逐渐减量。对脑脊液进行了VGKC抗体检测，检测结果显示LGI（富含亮氨酸的胶质瘤失活1）抗体呈阳性。根据美国血浆分离协会（ASFA）治疗神经综合征指南的建议，连续6天每天安排进行治疗性血浆置换（TPE）。
对诊断为LE且存在VGKC抗体的患者每天进行TPE已显示出在控制症状方面有快速改善。
