目的 探讨急性播散性脑脊髓炎(ADEM)合并抗接触蛋白关联蛋白2(Caspr 2)抗体自身免疫性脑炎的临 床特征。方法 回顾分析1例ADEM合并抗Caspr2抗体自身免疫性脑炎患儿的临床资料。结果 患儿,女性,7岁10月龄, 因发热伴精神差入院,病程中相继出现癫痫发作、嗜睡、昏迷、认知功能损害、精神性格改变、失眠、神经性疼痛等。头 颅磁共振(MRI)表现为T 2 WI、FLAIR序列双侧大脑半球内多发散在斑片状高信号影。病程第4天患儿脑脊液Caspr2 抗体阴性,病程第21天复查脑脊液Caspr 2抗体转为阳性,诊断为ADEM合并抗Caspr 2抗体自身免疫性脑炎。给予糖皮 质激素和免疫球蛋白冲击治疗,并加用血浆置换,患儿临床及头颅MRI恢复良好。结论 ADEM合并抗Caspr 2抗体自 身免疫性脑炎罕见,及时完善血液和脑脊液自身免疫性脑炎相关抗体、头颅MRI等相关辅助检查有利于及时诊治,改善 预后。
Objective To explore the clinical characteristics of acute disseminated encephalomyelitis (ADEM) with anti-contactin-associated protein-like 2 (Caspr 2 ) antibody associated autoimmune encephalitis. Methods The clinical data of ADEM complicated with anti-Caspr 2 antibody associated autoimmune encephalitis in a child were analyzed retrospectively. Results A 7 -year- and 10 -month-old girl was admitted to hospital due to fever and mental distress. During the course of the disease, she developed clinical symptoms including seizures, lethargy, coma, cognitive impairment, mental personality changes, insomnia, and neuropathic pain and so on. Cranial magnetic resonance imaging (MRI) showed patchy-like high-signal shadows in bilateral cerebral hemispheres with T 2 WI and FLAIR sequences. On the 4 th day of the disease course, the cerebrospinal fluid Caspr 2 antibody was negative, and the cerebrospinal fluid Caspr 2 antibody turned positive on the 21 st day of the disease course, and ADEM complicated with anti-Caspr 2 antibody associated autoimmune encephalitis was diagnosed. The clinical symptoms and cranial MRI of the children recovered well after the treatment with glucocorticoid and immunoglobulin plus plasmapheresis. Conclusions ADEM complicated with anti-Caspr 2 antibody associated autoimmune encephalitis is rare. Prompt completion of blood and cerebrospinal fluid autoimmune encephalitis-related antibody examinations, head MRI and other related auxiliary examinations are conducive to timely diagnosis and treatment and improvement of prognosis.