Department of Center for Psychosomatic Medicine, Sichuan Provincial Center for Mental Health, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, Chengdu, China.
Department of Neurology, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, No. 32 West Second Section of First Loop, Qingyang District, Chengdu City, Sichuan Province, China.
Neurol Sci. 2024 Jan;45(1):83-92. doi: 10.1007/s10072-023-07034-x. Epub 2023 Sep 18.
Previous studies suggest a relationship between central nervous system inflammatory demyelinating diseases and anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis. Also, the overlap between anti-NMDAR encephalitis and multiple sclerosis (MS) has been reported. However, the pathogenesis and clinical characteristics are still obscure.
A 33-year-old woman presented with diplopia and sensory ataxia at the onset. The cerebrospinal fluid (CSF) anti-NMDAR antibodies were positive (1:3.2), and nuclear magnetic resonance imaging (MRI) showed bilateral centrum ovale and lateral ventricle demyelinating lesions. Therefore, she was diagnosed with anti-NMDAR encephalitis. After administering intravenous immunoglobulin and oral prednisone, her lesions disappeared, and symptoms were relieved. The condition was maintained with a low dose of prednisone, but her lesions reappeared on MRI. Consequently, immunomodulatory therapy of mycophenolate mofetil was initiated. However, she developed dysarthria and right limb ataxia after 10 months with a positive CSF anti-NMDAR antibody (1:1) and positive oligoclonal band. The MRI showed symmetrical multiple demyelinating lesions. Considering the MS diagnosis, her neurological dysfunction again improved significantly after intravenous methylprednisolone. Unfortunately, her symptoms aggravated for the second time when teriflunomide was started. Finally, her condition was controlled again with oral prednisone.
Consistent with previous cases of overlapping anti-NMDAR encephalitis and MS, patients often show atypical symptoms on MRIs and immunological tests. The overlap cannot be arbitrarily treated because of the recurrence of previous diseases. Long-term follow-up, dynamic antibody monitoring, and MRI examination are crucial for these patients. The special dependency of the patient on glucocorticoids in this study has been rarely reported, which may guide the treatment of insensitivity to disease-modifying therapy in recurrent overlapping anti-NMDAR encephalitis and MS.
先前的研究表明中枢神经系统炎症性脱髓鞘疾病与抗 N-甲基-D-天冬氨酸受体（NMDAR）脑炎之间存在关联。此外，抗 NMDAR 脑炎与多发性硬化症（MS）之间也存在重叠。然而，其发病机制和临床特征仍不清楚。
一名 33 岁女性以复视和感觉共济失调为首发症状。脑脊液（CSF）抗 NMDAR 抗体阳性（1:3.2），磁共振成像（MRI）显示双侧脑干部和侧脑室脱髓鞘病变。因此，她被诊断为抗 NMDAR 脑炎。静脉注射免疫球蛋白和口服泼尼松后，她的病灶消失，症状缓解。病情用低剂量泼尼松维持，但 MRI 上再次出现病灶。随后开始霉酚酸酯免疫调节治疗。然而，10 个月后，她出现构音障碍和右侧肢体共济失调，CSF 抗 NMDAR 抗体（1:1）和寡克隆带阳性。MRI 显示对称多发脱髓鞘病变。考虑到 MS 的诊断，她再次接受静脉用甲基泼尼松龙治疗后神经功能障碍明显改善。不幸的是，当开始使用特立氟胺时，她的症状又加重了第二次。最后，她的病情再次通过口服泼尼松得到控制。
与先前重叠抗 NMDAR 脑炎和 MS 的病例一致，患者 MRI 和免疫检查通常表现出不典型症状。由于先前疾病的复发，不能随意重叠治疗。这些患者需要长期随访、动态抗体监测和 MRI 检查。本研究中患者对糖皮质激素的特殊依赖性很少有报道，这可能指导复发性重叠抗 NMDAR 脑炎和 MS 对疾病修饰治疗不敏感的治疗。
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