Celicanin M, Blaabjerg M, Maersk-Moller C, Beniczky S, Marner L, Thomsen C, Bach F W, Kondziella D, Andersen H, Somnier F, Illes Z, Pinborg L H
Department of Neurology, Bispebjerg Hospital, Copenhagen, Denmark.
Department of Neurology, Roskilde University Hospital, Roskilde, Denmark.
Eur J Neurol. 2017 Aug;24(8):999-1005. doi: 10.1111/ene.13324. Epub 2017 May 25.
The aim of this study was to describe clinical and paraclinical characteristics of all Danish patients who tested positive for anti-voltage-gated potassium channels (VGKC)-complex, anti-leucine-rich glioma-inactivated 1 (LGI1) and anti-contactin-associated protein-2 antibodies in the serum/cerebrospinal fluid between 2009 and 2013 with follow-up interviews in 2015 and 2016.
We evaluated antibody status, symptoms leading to testing, course of disease, suspected diagnosis and time of admission as well as diagnosis and treatment. All magnetic resonance imaging, electroencephalography and F-fluorodeoxyglucose positron emission tomography scans were re-evaluated by experts in the field.
A total of 28/192 patients tested positive for VGKC-complex antibodies by radioimmunoassay and indirect immunofluorescence; 17 had antibodies to LGI1 and 6/7 of the available cerebrospinal fluids from these patients were seropositive. These 17 patients all had a clinical phenotype appropriate to LGI1 antibodies. The remaining 11 were LGI1 negative (n = 4) or not tested (n = 7). Of these, two had a phenotype consistent with limbic encephalitis. The remaining phenotypes were Guillain-Barré syndrome, Creutzfeldt-Jakob disease, neuromyotonia and anti-N-methyl-D-aspartate receptor encephalitis. Magnetic resonance imaging abnormalities were demonstrated in 69% of the LGI1-positive patients. Two patients with normal magnetic resonance imaging demonstrated temporal lobe hypermetabolism using F-fluorodeoxyglucose positron emission tomography. Abnormal electroencephalography recordings were found in 86% of the patients. Upon follow-up (median 3.2 years), the median modified Rankin Scale score of anti-LGI1-positive patients was 2 and only two patients reported seizures in the past year.
Patients diagnosed with anti-LGI1 autoimmune encephalitis increased significantly from 2009 to 2014, probably due to increased awareness. In contrast to seropositive anti-VGKC-complex patients, all anti-LGI1-positive patients presented with a classical limbic encephalitis. The majority of patients recovered well.
本研究旨在描述2009年至2013年间丹麦所有血清/脑脊液中抗电压门控钾通道（VGKC）复合物、抗富含亮氨酸胶质瘤失活1蛋白（LGI1）及抗接触蛋白相关蛋白2抗体检测呈阳性的患者的临床和副临床特征，并于2015年和2016年进行随访访谈。
我们评估了抗体状态、导致检测的症状、病程、疑似诊断及入院时间以及诊断和治疗情况。所有磁共振成像、脑电图和氟脱氧葡萄糖正电子发射断层扫描均由该领域的专家重新评估。
共有28/192例患者通过放射免疫分析和间接免疫荧光法检测VGKC复合物抗体呈阳性；17例有LGI1抗体，这些患者中7份可用脑脊液中的6份血清学呈阳性。这17例患者均具有与LGI1抗体相符的临床表型。其余11例为LGI1阴性（n = 4）或未检测（n = 7）。其中，2例具有与边缘性脑炎相符的表型。其余表型为格林-巴利综合征、克雅氏病（Creutzfeldt-Jakob disease）、神经性肌强直和抗N-甲基-D-天冬氨酸受体脑炎。69%的LGI1阳性患者磁共振成像有异常表现。2例磁共振成像正常的患者经氟脱氧葡萄糖正电子发射断层扫描显示颞叶代谢亢进。86%的患者脑电图记录异常。随访时（中位时间3.2年），抗LGI1阳性患者的改良Rankin量表评分中位数为2，过去一年仅有2例患者报告有癫痫发作。
2009年至2014年期间，诊断为抗LGI1自身免疫性脑炎的患者显著增加，可能是由于认识提高。与血清学阳性的抗VGKC复合物患者不同，所有抗LGI1阳性患者均表现为典型的边缘性脑炎。大多数患者恢复良好。
