中华神经科杂志
中華神經科雜誌
중화신경과잡지
Chinese Journal of Neurology
2014年
7期
474-481
,共8页
刘磊%宋兆慧%郭晶%代飞飞%朴月善%许春伶%王瑞金%姜彬%张丽燕
劉磊%宋兆慧%郭晶%代飛飛%樸月善%許春伶%王瑞金%薑彬%張麗燕
류뢰%송조혜%곽정%대비비%박월선%허춘령%왕서금%강빈%장려연
抗N-甲基-D-天冬氨酸受体脑炎%受体,N-甲基-D-天冬氨酸%自身抗体%抗体,抗核%日常生活活动%疾病严重程度指数
抗N-甲基-D-天鼕氨痠受體腦炎%受體,N-甲基-D-天鼕氨痠%自身抗體%抗體,抗覈%日常生活活動%疾病嚴重程度指數
항N-갑기-D-천동안산수체뇌염%수체,N-갑기-D-천동안산%자신항체%항체,항핵%일상생활활동%질병엄중정도지수
Anti-N-methyl-D-aspartate receptor encephalitis%Receptors,N-methyl-D-aspartate%Autoantibodies%Antibodies,antinuclear%Activities of daily living%Severity of illness index
目的 分析中国人抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床及辅助检查特点.方法 对255例临床疑似脑炎患者的血清及脑脊液行抗NMDAR-IgG检测.结合临床表现确诊抗NMDAR脑炎患者,收集其临床及辅助检查资料,并按年龄分为＜12岁,12～17岁以及＞17岁3组.筛查上述患者标本中其他自身免疫及感染相关抗体,并对25例确诊半年以上患者进行随访.结果 自255例中共确诊45例抗NMDAR脑炎患者(占17.6％),男∶女为24∶21,中位年龄10岁,3例患者合并可疑盆腔占位.发病时间多集中在5月、6月、9月及10月.3个年龄组首发症状不同,发病1个月内主要症状亦不相同.26例患者血清和脑脊液抗NMDAR-IgG均阳性,17例患者仅脑脊液IgG阳性,2例患者仅血清IgG阳性.脑脊液抗NMDAR-IgA阳性患者的脑脊液抗NMDAR-IgG滴度高于脑脊液IgA阴性患者.部分患者血清中存在其他自身免疫抗体.6例患者血清风疹病毒IgM阳性,1例血清单核细胞增多性李斯特菌IgM阳性.抗NMDAR脑炎患者脑脊液细胞学演变过程与病毒性脑炎类似.45例患者均行脑电图检查,41例表现异常,主要为广泛慢波,1例出现“极端δ刷”(extreme delta brush).15例患者头颅MRI表现异常.全部患者均接受一线免疫治疗,9例患者接受二线免疫治疗.随访25例确诊半年以上患者,发现脑脊液抗体滴度与患者预后无关.改良Rankin量表(mRS)评分≥5分及mRS评分≤2分患者的中位确诊时间分别为35 d及17 d,差异有统计学意义(Z=-2.096,P=0.036).结论 我国抗NMDAR脑炎发病率被低估.本组抗NMDAR脑炎患者相对西方患者具有男性比例高、年轻患者多、合并肿瘤者少等特点.行为异常、运动障碍、认知缺陷、癫痫发作及自主神经功能障碍为本组患者的五大早期表现.抗NMDAR-IgG可能多合成自鞘内.一些感染因子可能参与本病的发生.本病无特异影像学表现.早期诊断对预后十分重要.
目的 分析中國人抗N-甲基-D-天鼕氨痠受體(NMDAR)腦炎的臨床及輔助檢查特點.方法 對255例臨床疑似腦炎患者的血清及腦脊液行抗NMDAR-IgG檢測.結閤臨床錶現確診抗NMDAR腦炎患者,收集其臨床及輔助檢查資料,併按年齡分為＜12歲,12～17歲以及＞17歲3組.篩查上述患者標本中其他自身免疫及感染相關抗體,併對25例確診半年以上患者進行隨訪.結果 自255例中共確診45例抗NMDAR腦炎患者(佔17.6％),男∶女為24∶21,中位年齡10歲,3例患者閤併可疑盆腔佔位.髮病時間多集中在5月、6月、9月及10月.3箇年齡組首髮癥狀不同,髮病1箇月內主要癥狀亦不相同.26例患者血清和腦脊液抗NMDAR-IgG均暘性,17例患者僅腦脊液IgG暘性,2例患者僅血清IgG暘性.腦脊液抗NMDAR-IgA暘性患者的腦脊液抗NMDAR-IgG滴度高于腦脊液IgA陰性患者.部分患者血清中存在其他自身免疫抗體.6例患者血清風疹病毒IgM暘性,1例血清單覈細胞增多性李斯特菌IgM暘性.抗NMDAR腦炎患者腦脊液細胞學縯變過程與病毒性腦炎類似.45例患者均行腦電圖檢查,41例錶現異常,主要為廣汎慢波,1例齣現“極耑δ刷”(extreme delta brush).15例患者頭顱MRI錶現異常.全部患者均接受一線免疫治療,9例患者接受二線免疫治療.隨訪25例確診半年以上患者,髮現腦脊液抗體滴度與患者預後無關.改良Rankin量錶(mRS)評分≥5分及mRS評分≤2分患者的中位確診時間分彆為35 d及17 d,差異有統計學意義(Z=-2.096,P=0.036).結論 我國抗NMDAR腦炎髮病率被低估.本組抗NMDAR腦炎患者相對西方患者具有男性比例高、年輕患者多、閤併腫瘤者少等特點.行為異常、運動障礙、認知缺陷、癲癇髮作及自主神經功能障礙為本組患者的五大早期錶現.抗NMDAR-IgG可能多閤成自鞘內.一些感染因子可能參與本病的髮生.本病無特異影像學錶現.早期診斷對預後十分重要.
목적 분석중국인항N-갑기-D-천동안산수체(NMDAR)뇌염적림상급보조검사특점.방법 대255례림상의사뇌염환자적혈청급뇌척액행항NMDAR-IgG검측.결합림상표현학진항NMDAR뇌염환자,수집기림상급보조검사자료,병안년령분위＜12세,12～17세이급＞17세3조.사사상술환자표본중기타자신면역급감염상관항체,병대25례학진반년이상환자진행수방.결과 자255례중공학진45례항NMDAR뇌염환자(점17.6％),남∶녀위24∶21,중위년령10세,3례환자합병가의분강점위.발병시간다집중재5월、6월、9월급10월.3개년령조수발증상불동,발병1개월내주요증상역불상동.26례환자혈청화뇌척액항NMDAR-IgG균양성,17례환자부뇌척액IgG양성,2례환자부혈청IgG양성.뇌척액항NMDAR-IgA양성환자적뇌척액항NMDAR-IgG적도고우뇌척액IgA음성환자.부분환자혈청중존재기타자신면역항체.6례환자혈청풍진병독IgM양성,1례혈청단핵세포증다성리사특균IgM양성.항NMDAR뇌염환자뇌척액세포학연변과정여병독성뇌염유사.45례환자균행뇌전도검사,41례표현이상,주요위엄범만파,1례출현“겁단δ쇄”(extreme delta brush).15례환자두로MRI표현이상.전부환자균접수일선면역치료,9례환자접수이선면역치료.수방25례학진반년이상환자,발현뇌척액항체적도여환자예후무관.개량Rankin량표(mRS)평분≥5분급mRS평분≤2분환자적중위학진시간분별위35 d급17 d,차이유통계학의의(Z=-2.096,P=0.036).결론 아국항NMDAR뇌염발병솔피저고.본조항NMDAR뇌염환자상대서방환자구유남성비례고、년경환자다、합병종류자소등특점.행위이상、운동장애、인지결함、전간발작급자주신경공능장애위본조환자적오대조기표현.항NMDAR-IgG가능다합성자초내.일사감염인자가능삼여본병적발생.본병무특이영상학표현.조기진단대예후십분중요.
Objective To analyse the clinical features of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in Chinese patients.Methods In this study,we tested the presence of antiNMDAR-IgG in serum and cerebrospinal fluid (CSF) of 255 clinical suspected encephalitis patients.AntiNMDAR encephalitis was diagnosed according to the clinical features and anti-NMDAR-IgG positivity.Clinical data of the diagnosed patients were collected.Then patients were divided into three groups according to ages (＜ 12 years old,12-17 years old and ＞ 17 years old).Their samples were sent out for further 10.3760/cma.j.issn.1006-7876.2014.07.009autoimmune and infectious antibodies scanning.Twenty-five patients diagnosed over 6 months were followed up.Results Anti-NMDAR encephalitis was found in 45 of 255 patients (17.6％),with a male:female ratio of 24∶ 21.Median age at disease onset was 10 years.Three patients had suspicious pelvic lesions.The highest incidence of symptoms onset was presented in May,June,September and October.The frequencies of the first symptoms were different among three age groups.During the first month of the disease,the frequencies of symptoms were also different from one another in three age groups.Twenty-six patients were found anti-NMDAR-IgG positive in both sera and CSF,17 patients only CSF IgG positive and 2 patients only sera IgG positive.Titers of CSF anti-NMDAR-IgG were higher in the CSF anti-NMDAR-IgA positive patients than those negative.Some patients were found with other autoimmune antibodies.Serum anti-rubella virus IgM was found positive in 6 patients and serum anti-Listeria monocytogenes IgM positive in 1 patient.Evolution of CSF cytology in patients with anti-NMDAR encephalitis was similar to those with viral encephalitis.Electroencephalograms were found abnormal in 41 patients.Universal slow wave was the most common finding and extreme delta brush was noted in one patient.Head MR images were abnormal in 15 patients.All the patients received first-line immunotherapies,and 9 patients also received second-line immunotherapies.During the follow-up of 25 patients who were diagnosed over 6 months,CSF anti-NMDAR-IgG titers were not relevant with the prognosis.The median diagnostic intervals of mRS score ≥ 5 and mRS score ≤2 were 35 days and 17 days,respectively (Z =-2.096 P =0.036).Conclusions The prevalence of anti-NMDAR encephalitis has been underestimated in China.This cohort has higher male to female ratio,younger age and less proportion of tumor than the western patients.Behavioral changes,movement disorders,cognitive deficits,seizures and autonomic symptoms are the top 5 early clinical features in this cohort.The majority of anti-NMDAR-IgG may be intrathecally synthesized.Some infectious agents may contribute to the disease.Non-specific alterations of cranial magnetic resonance images were found in our research.Early diagnosis is vital for prognosis.
