自身免疫性痴呆八例临床特点分析  

Analysis of clinical features for 8 patients with autoimmune dementia

作　　者：张伟赫[1] 矫毓娟[1] 李旭东[1] 贾树红[1] 王康[1] 刘尊敬[1] 焦劲松[1] 

机构地区：[1]中日友好医院神经内科,北京100029

出　　处：《中华医学杂志》2014年第5期359-363,共5页National Medical Journal of China

摘　　要：目的 探讨自身免疫性痴呆的临床特点和治疗措施,提高对本病的认识.方法 对2011年3月至2013年5月中日友好医院收治的8例自身免疫性痴呆患者的临床、治疗以及预后进行回顾性分析.结果 男3例,女5例,年龄45~72岁.急性起病3例,亚急性起病1例,慢性起病4例;波动性病程6例.多发性硬化3例,副肿瘤性边缘叶脑炎2例,桥本脑病、未分类自身免疫性脑病以及显微镜下多血管炎各1例;8例患者均伴不同程度记忆力下降,病程中均无谵妄;3例患者伴癫痫发作,2例伴顽固性低钠血症;4例检测到自身免疫抗体谱或副肿瘤抗体谱阳性;7例呈炎性脑脊液表现;4例脑电图表现为单或双侧颞叶为主慢波或尖慢复合波;8例头颅MRI均有异常表现;2例伴桥本甲状腺炎,1例伴小细胞肺癌;全部患者应用免疫治疗或抗肿瘤治疗均获得不同程度缓解.结论 自身免疫性痴呆病因复杂,临床表现为迅速进展性、波动性认知功能障碍,可合并顽固性癫痫和低钠血症,血清自身抗体、炎性脑脊液、异常脑电图以及头颅影像学改变有助于诊断,对各种免疫治疗措施有效.Objective To explore the clinical features and therapeutic profiles of autoimmune dementia. Methods Eight hospitalized patients with autoimmune dementia during March 2011 and May 2013 were recruited and retrospectively analyzed for clinical features, as well as therapeutic and prognosis profiles. Results There were 3 males and 5 females with a onset age range of 45 - 72 years. Their onsets varied from acute （ n = 3 ） , subacute （ n = 1 ） to chronic （ n = 4）. Six of them had a fluctuating course. The diagnoses were multiple sclerosis （ n = 3 ） , paraneoplastic limbic encephalitis （ n = 2 ） and Hashimoto＇ s encephalopathy （ n = 1 ）, microscopic polyangiitis （ n = 1 ） and unclassified autoimmune encephalopathy （ n = 1 ）. Progressive memory loss without delirium was the main symptom. In addition, 3 patients suffered epilepsy, 2 with intractable hyponatremia, 4 with positive serum autoimmune or paraneoplastic antibodies, 7 with inflammatory cerebrospinal fluid, 4 with abnormal electroencephalography （EEG） and 8 with various changes on brain magnetic resonance imaging （MRI）. Two patients had concurrent Hashimoto＇s thyroiditis and another with small cell lung cancer. All patients improved after treatment with immunological and antineoplastic therapies. Conclusion Autoimmune dementia has complex causes with a rapidly progressive and fluctuating course. The coexisting conditions include epilepsy, hyponatremia, organ-specific autoimmunity, inflammatory spinal fluid with abnormal EEG and brain MRI findings. Immunotberapy is recommended.

关 键 词：痴呆 自身免疫性 边缘叶脑炎 桥本脑病 多发性硬化 显微镜下多血管炎 

分 类 号：R741[医药卫生—神经病学与精神病学]