Chen Jingjiong, Tang Xin, Wang Linran, Guo Dandan, Cao Li, Lu Kaili
Department of Neurology, Shanghai Jiao Tong University School of Medicine Affiliated Sixth People's Hospital, Shanghai Neurological Rare Disease Biobank and Precision Diagnostic Technical Service Platform, Shanghai, China.
Front Immunol. 2025 May 30;16:1568005. doi: 10.3389/fimmu.2025.1568005. eCollection 2025.
Anti-metabotropic glutamate receptor 5 (mGluR5) encephalitis is a rare autoimmune disorder characterized by varied neurological symptoms, often associated with limbic syndromes. We report the case of a 57-year-old female who presented with obvious ataxia, and then cognitive impairment and behavioral changes. MRI revealed cerebellar dentate nucleus and basal ganglia lesions with enhancement, which spontaneously regressed on follow-up imaging-a rare phenomenon in mGluR5 encephalitis. Diagnosis was confirmed by the presence of serum mGluR5 antibodies. Extensive evaluations excluded underlying malignancies. Immunotherapy, including corticosteroids and intravenous immunoglobulin, led to significant clinical and radiological improvement. This case highlights the unique cerebellar dentate nucleus and basal ganglia involvement and spontaneous partial reduction of lesions in mGluR5 encephalitis, expanding the clinical spectrum of this condition. Early recognition and immunotherapy are crucial for favorable outcomes. Further studies are needed to elucidate the mechanisms behind spontaneous partial lesion regression in autoimmune encephalitis.
抗代谢型谷氨酸受体5(mGluR5)脑炎是一种罕见的自身免疫性疾病,其特征为多种神经症状,常与边缘叶综合征相关。我们报告了一例57岁女性患者,该患者最初表现为明显的共济失调,随后出现认知障碍和行为改变。磁共振成像(MRI)显示小脑齿状核和基底神经节病变并伴有强化,而在后续成像中这些病变自发消退,这在mGluR5脑炎中是一种罕见现象。血清mGluR5抗体的存在证实了诊断。广泛评估排除了潜在恶性肿瘤。包括皮质类固醇和静脉注射免疫球蛋白在内的免疫治疗使临床和影像学有显著改善。该病例突出了mGluR5脑炎中独特的小脑齿状核和基底神经节受累以及病变的自发部分消退,拓宽了该病的临床谱。早期识别和免疫治疗对取得良好预后至关重要。需要进一步研究以阐明自身免疫性脑炎中病变自发部分消退背后的机制。