| Objective:Investigate the clinical characteristics,auxiliary examination,diagnosis,treatment of autoimmune encephalitis and prognosis in children.Methods:Make a retrospective analysis of the cases of 45 children with autoi mmune encephalitis which is collected from the First Affiliated Hospital of Kun ming Medical University and cooperative Hospital from January 2012 to March 2018,observe and contrast the clinical characteristics of children with autoimmun e encephalitis in each group.the characteristics of cerebrospinal fluid(CSF),(CS F)autoimmune encephalitis antibody,MRI in the cranial spinal cord,and electroe ncephalogram(EEG),immunotherapy effect,and traced the prognosis.Results:1.Classification of autoimmune encephalitis,the first group:7 cases of a nti-NMDA receptor encephalitis.The second group:8 cases of autoantibody-negat ive autoimmune encephalitis,The third group:19 cases of acute disseminated en cephalomyelitis(ADEM);The fourth group:other autoimmune encepha(?)tis:2 case s of Hashimoto’s encephalopathy,2 cases of primary immune vascu(?)tis,2 cases of rheumatic chorea,3 cases of neuromyelitis optica,1 cases of lupus encephalitis,and 1 cases of Rasmussen’s encephalitis.2.Clinical manifestations:epileptic seizures 13/45(29%),most of the form of co mprehensive seizures;abnormal mental behavior in 18/45 cases(40%),manifest ed as irritability,indifference,nonsense,a part of the hallucination of auditory ha llucination;language disorders 22/45 cases(49%),manifested as dysarthria and s1 ow language;Level of consciousness decline 13/45 cases(29%),manifested as S omnolence and coma.3.The number of cerebrospinal fluid cells increased in 17/44(39%),with lymph ocytes increased mainly,protein increased in 10/44 cases(22%),1gG index incre ased in 15/27 cases(55%),and the positive rate of autoimmune encephalitis anti body was 7/25(28%).4.Abnormal electroencephalogram in 28/34 cases(82%),manifested as non-spe cific background rhythm slowing down in 25/34(74%),epileptiform discharge i n 9/34 cases(26%),focal slow wave in 7/34(21%).No "extreme delta brush"was found in this case.5.All patients were given first-line treatment(methylprednisolone intravenous dri p,Immune globulin 2g/kg).and 2 patients were treated with second-line therapy(rituximab、Cyclopho sphami de).6.The improved Rankin scale was used to evaluate the prognosis:Good prognosis(MRS≤2)37/45(82%),death was 2 cases,mortality was 4%,7 cases of anti NMDAR encephalitis,recurrence 3 cases,recurrence rate 43%.Conclusion:1.In the early stage,children with epileptic seizures and abnormal mental behavior need to be alert to autoimmune encephalitis,especially anti-NMDAR encephalitis.2.Antibody positive rate of Autoimmune encephalitis is about 1/3,but antibody negative also can’t exclude autoimmune encephalitis,the clinical manifestations of children with antibody negative autoimmune encephalitis are consistent with antibody positive autoimmune encephalitis.3.The abnormal rate of head MRI was 73%,but the anti NMDAR encephalitis and antibody negative AE,MRI abnormal rate was 47%,all showed cortical and subcortical lesions.The abnormal rate of EEG was 82%,which was mainly slowed down by non-specific background rhythm,partly with epileptiform discharges and focal slow waves.In combination with three major signs of autoimmune encephalitis,acute or subacute seizures,language disorders and abnormal mental behavior,abnormal EEG may be an important indicator window.5.The relapse rate of children with NMDAR encephalitis is 43%.Repeated first-line treatment after relapse still has very good curative effect. |
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