Xia Junyang, Yin Xiaoming, Zhu Mingqin, Cao Jie, Song Xiaonan
Department of Neurology and Neuroscience Center, First Hospital of Jilin University, Changchun, Jilin, China.
Medicine (Baltimore). 2018 Jan;97(3):e9574. doi: 10.1097/MD.0000000000009574.
Autoimmune encephalitis (AE) is a heterogeneous group of recently identified disorders. Despite severe and even prolonged neurologic deficits, dramatic improvements may occur with proper immunotherapy in some patients with AE. Antineuronal antibodies have been discovered in patients' serum and cerebrospinal fluid (CSF). However, AE with multiple antineuronal antibodies is rare. To date, there are no published reports of AE with both anti-γ-aminobutyric acid B receptor (GABABR) and anticollapsin response-mediator protein 5 (CV2) antibodies.
We describe a 46-year-old man who presented with seizures, working memory deficits, and visual hallucinations. We detected anti-CV2 and anti-GABABR antibodies in his serum and CSF. Brain magnetic resonance imaging (MRI) revealed patchy abnormal signals in his left temporal lobe and hippocampus. The patient's symptoms improved after receiving intravenous immunoglobulin injections and glucocorticoids, but his condition relapsed within 4 months, and he was readmitted to our hospital. Repeated MRI scans revealed new lesions in his right temporal lobe and hippocampus.
The AE diagnosis was established from the results of the preliminary physical examination, the laboratory tests, and the imaging findings.
The patient received intravenous immunoglobulins and glucocorticoids.
We followed the patient for 9 months from the date of the patient's second hospital discharge. He experienced no seizures during this period, but his short-term memory deficits and visual hallucinations were not completely alleviated.
Coexisting anti-CV2 and anti-GABABR antibodies may have synergistic effects and worsen the clinical syndrome. AE with multiple antineuronal antibodies may be relapse-prone. Further studies investigating the relationship between anti-CV2 and anti-GABABR antibodies are warranted.
自身免疫性脑炎（AE）是一组最近才被确认的异质性疾病。尽管存在严重甚至长期的神经功能缺损，但部分AE患者通过适当的免疫治疗可能会有显著改善。已在患者血清和脑脊液（CSF）中发现抗神经元抗体。然而，伴有多种抗神经元抗体的AE较为罕见。迄今为止，尚无关于同时存在抗γ-氨基丁酸B受体（GABABR）和抗塌陷反应介导蛋白5（CV2）抗体的AE的报道。
我们描述了一名46岁男性，他出现癫痫发作、工作记忆缺陷和视幻觉。我们在他的血清和脑脊液中检测到抗CV2和抗GABABR抗体。脑磁共振成像（MRI）显示其左侧颞叶和海马有斑片状异常信号。患者在接受静脉注射免疫球蛋白和糖皮质激素治疗后症状改善，但4个月内病情复发，再次入院。重复MRI扫描显示其右侧颞叶和海马出现新病灶。
根据初步体格检查、实验室检查和影像学检查结果确诊为AE。
患者接受了静脉注射免疫球蛋白和糖皮质激素治疗。
从患者第二次出院之日起，我们对其进行了9个月的随访。在此期间他未出现癫痫发作，但其短期记忆缺陷和视幻觉未完全缓解。
同时存在抗CV2和抗GABABR抗体可能具有协同作用并使临床综合征恶化。伴有多种抗神经元抗体的AE可能易于复发。有必要进一步研究抗CV2和抗GABABR抗体之间的关系。
