Department of Neurology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.
Department of Neurology, Affiliated Hospital of Chifeng University, Chifeng, Inner Mongolia, China.
Brain Behav. 2023 May;13(5):e3003. doi: 10.1002/brb3.3003. Epub 2023 Apr 14.
To describe the clinical and radiological characteristics of anti-metabotropic glutamate receptor 5 (mGluR5) encephalitis.
We reviewed the clinical data of five patients with anti-mGluR5 encephalitis, and performed a literature review.
The five cases included a 52-year-old man who developed a biphasic course of anti-mGluR5 encephalitis after herpes simplex encephalitis, a 22-year-old woman who showed bilateral basal ganglia lesions on brain magnetic resonance imaging (MRI), and a 36-year-old man with mixed aphasia and generalized tonic-clonic seizures, a 51-year-old man presented with personality changes, hallucinations, delusions, sleeping disorders and a 58-year-old man with short-term memory deficits and absence seizures.. There are 16 reported cases of anti-mGluR5 encephalitis worldwide. Of all 21 patients, with a median onset age of 35 years old, the main neurological symptoms were cognitive impairment (85.7%, 18/21), psychiatric or behavior problems (76.2%, 16/21), seizures (57.1%, 12/21), sleeping disorders (52.4%, 11/21), different degrees of decreased consciousness (42.9%, 9/21), and movement disorders (23.8%, 5/21). Brain MRI was normal in 11 of 21 patients. Lesions of the limbic lobes were presented in 5 patients, while involvement of other extralimbic regions was also reported. Seven of 21 (33.3%) cases were combined with tumors. Elevated white blood cell counts or specific oligoclonal IgG bands in the cerebrospinal fluid were found in 18 of 21 patients, with marked improvements observed after immunotherapy.
Patients with anti-mGluR5 encephalitis typically present with diffuse, rather than purely limbic, encephalitis. Anti-mGluR5 encephalitis can be triggered by herpes simplex encephalitis. The risk of a combined tumor may be reduced in anti-mGluR5 encephalitis patients.
描述抗代谢型谷氨酸受体 5(mGluR5)脑炎的临床和影像学特征。
我们回顾了五例抗 mGluR5 脑炎患者的临床资料,并进行了文献复习。
这五例患者包括一例 52 岁男性,在单纯疱疹脑炎后出现抗 mGluR5 脑炎的双相病程;一例 22 岁女性,脑磁共振成像(MRI)显示双侧基底节病变;一例 36 岁男性,表现为混合性失语和全面强直阵挛发作;一例 51 岁男性,表现为人格改变、幻觉、妄想、睡眠障碍;一例 58 岁男性,表现为短期记忆缺失和失神发作。全世界共报道了 16 例抗 mGluR5 脑炎病例。在所有 21 例患者中,中位发病年龄为 35 岁,主要的神经系统症状为认知障碍(85.7%,18/21)、精神或行为问题(76.2%,16/21)、癫痫发作(57.1%,12/21)、睡眠障碍(52.4%,11/21)、不同程度的意识障碍(42.9%,9/21)和运动障碍(23.8%,5/21)。21 例患者中 11 例脑 MRI 正常。5 例患者存在边缘叶病变,也有报道存在其他边缘外区域受累。21 例中有 7 例(33.3%)合并肿瘤。21 例中有 18 例患者脑脊液白细胞计数升高或存在特异性寡克隆 IgG 带,免疫治疗后明显改善。
抗 mGluR5 脑炎患者通常表现为弥漫性而非单纯边缘性脑炎。抗 mGluR5 脑炎可由单纯疱疹脑炎引发。抗 mGluR5 脑炎患者合并肿瘤的风险可能降低。