Department of Neurosurgery, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
Al-Zahra Research Institute, Isfahan University of Medical Sciences, Isfahan, Iran.
Neurol Sci. 2022 Feb;43(2):1239-1248. doi: 10.1007/s10072-021-05400-1. Epub 2021 Jul 30.
Even within the most populous countries in the Middle East, such as Iran, autoimmune encephalitis cases have been rarely reported.
We aimed to describe the demographic, clinical, and paraclinical characteristics of Iranian patients with autoimmune encephalitis positive for anti-neuronal autoantibodies.
This cross-sectional study included all patients diagnosed with autoimmune encephalitis and referred to our hospital, in Isfahan, Iran, from March 2016 to May 2020. Patients' demographic, clinical, laboratory, radiological, and electroencephalographic features were obtained from their medical records.
We identified a total of 39 (21 females, 53.8%) patients with autoimmune encephalitis (mean age = 34.9 ± 12.8 years). The most commonly detected antibody was anti-NMDAR (n = 26, 66.7%), followed by anti-GABAR (n = 8, 20.5%), anti-Zic4 (n = 4, 10.3%), and anti-GAD65 (n = 1, 2.6%) antibodies, in descending order of frequency. Two anti-NMDAR-positive patients had a history of systemic lupus erythematosus (SLE), and four had a prior history of herpes simplex encephalitis. Clinical presentations in patients positive for anti-Zic4 antibodies included cognitive decline (n = 4, 100%), seizures (n = 3, 75%), parkinsonism (n = 1, 25%), and stiff-person syndrome (n = 1, 25%).
This was the first case series of Iranian patients with autoimmune encephalitis with some interesting observations, including SLE-associated anti-NMDAR encephalitis, as well as an unusual concurrence of anti-Zic4 antibody positivity and cognitive problems, seizures, parkinsonism, and stiff-person syndrome.
即使在中东人口最多的国家，如伊朗，自身免疫性脑炎的病例也很少报告。
我们旨在描述伊朗抗神经元自身抗体阳性自身免疫性脑炎患者的人口统计学、临床和临床前特征。
这项横断面研究纳入了 2016 年 3 月至 2020 年 5 月期间所有被诊断为自身免疫性脑炎并转诊至伊朗伊斯法罕医院的患者。从患者病历中获取了他们的人口统计学、临床、实验室、影像学和脑电图特征。
我们共发现了 39 名（21 名女性，53.8%）自身免疫性脑炎患者（平均年龄 34.9±12.8 岁）。最常检测到的抗体是抗 NMDAR（n=26，66.7%），其次是抗 GABAR（n=8，20.5%）、抗 Zic4（n=4，10.3%）和抗 GAD65（n=1，2.6%）抗体，依次递减。两名抗 NMDAR 阳性患者有系统性红斑狼疮（SLE）病史，4 名患者有单纯疱疹性脑炎病史。抗 Zic4 抗体阳性患者的临床表现包括认知能力下降（n=4，100%）、癫痫发作（n=3，75%）、帕金森病（n=1，25%）和僵人综合征（n=1，25%）。
这是伊朗自身免疫性脑炎患者的首个病例系列研究，有一些有趣的观察结果，包括与 SLE 相关的抗 NMDAR 脑炎，以及抗 Zic4 抗体阳性与认知问题、癫痫发作、帕金森病和僵人综合征的不常见共存。
