| Objective:To investigate the clinical features of anti-NMDA receptor encephalitis and the value of rituximab in the treatment of anti-NMDA receptor encephalitisMethods:1.36 cases of children who were diagnosed of anti-NMDA receptor encephalitis at the Shandong University Qilu Hospital from June 2014 to June 2016 were selected as the research subjects,and clinical data of patients,such as signs and symptoms,auxiliary examination,treatment and prognosis,were retrospectively analyzed.2.According to the state of consciousness,the patients were divided into the consciousness group and the unconsciousness group,and age,mRS scores and GSC scores before and after treatment,length of stay between the two groups were compared,to analyze clinical features and efficacy of the two groups.3.According to the administration with rituximab or not,the children were divided into the non-rituximab group(the control group)and the rituximab group(the treatment group),to analyze clinical and auxiliary data of the two groups.4.mRS scores and serum immunoglobulin IgG,IgA,IgM and CD3+,CD4+,CD8+,CD 19+ in the peripheral blood of the rituximab group before the treatment and the second month,the first year after the treatment were retrospectively analyzed,to analyze the efficacy and safety of rituximab in the treatment of anti NMDAR receptor encephalitis.Results:1.36 patients with anti NMDAR receptor encephalitis,including 16 males and 20 females,presented following clinical manifestations:(1)15 cases presented fever,headache and other prodromal symptoms(41.67%);(2)35 cases demonstrated abnormal behaviors(97.22%);(3)31 cases presented movement disorders(86.11%);(4)30 cases demonstrated epilepsy(83.33%);(5)10 cases presented dysfunction of autonomic nervous system(27.78%),including sinus tachycardia in 5 cases,bradycardia in 1 case,right bundle branch block in 1 case,increased sweating and saliva secretion in 4 cases;(6)15 cases exhibited decreased consciousness(41.67%);(7)10 cases showed speech impediment(27.78%);(8)9 cases showed cognitive impairment(25%);(9)10 cases presented sleep disorders(27.78%).2.Among 36 patients with anti NMDAR receptor encephalitis,21 cases were classified into the non-consciousness group and 15 cases into the consciousness group.The non-consciousness group and consciousness group were aged at 5.2±3.25 and 6.1±2.63 years old,respectively,the difference being statistically-significant(P=0.32,P>0.05),suggesting that age and the severity of anti NMDAR receptor encephalitis are not correlated.mRS scores of the two groups before treatment:3.41 ±0.65 points in the unconsciousness group and 4.91 ±0.32 points in the consciousness group,the difference being statistically significant(P<0.05).mRS scores of the two groups after treatment,0.8±0.58 points in the unconsciousness group and 2.73±1.49 points in the consciousness group,the difference between the two groups being statistically significant(P<0.05).mRS scores of the two groups after treatment decreased significantly at 0.8±0.58 and 2.73±1.49,respectively,(P<0.05);GSC scores:15 points in the unconsciousness group and 5.92±2.31 points in the consciousness group,and the difference was statistically significant(P<0.05).The average length of stay of the unconsciousness group and the consciousness group were 45.58±32.21 days and 63.85±23.53 days,respectively,(P<0.05),the difference being statistically significant.3.Comparison between the control group and the treatment group:29 of 36 cases were treated with first-line drugs(large dose methylprednisolone pulse therapy and intravenous immunoglobulin),and 7 cases of patients with anti NMDAR receptor encephalitis who poorly responded to first-line immunotherapy were treated with second-line rituximab immunotherapy.The non-rituximab group and the rituximab group was aged at 6.2 ±2.17 and 5.4 ± 1.21 years old,respectively,the difference being not statistically significant(P=0.329,P>0.05).1 case in the control group and 5 cases in the treatment group presented the first symptom of consciousness disorder,and the difference was statistically significant(P<0.05);7 cases in the control group and 7 cases in the treatment group presented the first symptom of mental and behavior disorders,the difference being statistically significant(P<0.05);and the control group and the treatment group had no significant difference in the number of patients presenting the first symptoms of memory decline,sleep disorders,epilepsy seizures,movement disorders,language disorder(p>0.05).In addition,the total number of clinical symptoms lasting for less than 2 weeks in the control group and the treatment group were 1.1±0.4 and 5.1 ±0.5,respectively,and the difference was statistically significant(P<0.05).Peak mRS score in the course of the disease was 2.2±0.74 in the control group and 4.9±0.66 in the treatment group,the difference being statistically significant(P<0.05).The effective days of first-line immunotherapy and rituximab were 20.6±14.14 days in the control group and 10.1±4.93 days in the treatment group,the difference being statistically significant(P<0.05).mRS scores<2 took place within 68 ±21.41 days in the control group and 49.5± 17.25 days in the treatment group,P=0.54,difference being not statistically significant.In the first year of follow-up visit,6 children in the control group relapsed,and there was no recurrence in the treatment group.The difference between the two groups was statistically significant(P<0.05).4.The score of the patients in this group was 5 points,2.7±1.25 points,1.0±0.5 points and 0 point before the administration with rituximab,4 weeks,2 months and 1 year after the administration with rituximab,respectively.Serum IgG?IgA?IgM?CD3+?CD4+?CD8+ of patients before and after treatment were not statistically significant;CD 19+of patients decreased significantly after treatment(53.52±6.52%vs 0.21±0.14%),P<0.05,and the difference was statistically significant.Conclusions:1.Common clinical symptoms of children with anti-NMDAR receptor encephalitis include epilepsy,dyskinesia,mental and behavioral abnormalities,decreased consciousness,autonomic nervous dysfunction,sleep disorders,language disorders and cognitive decline.2.mRS and GSC scores can be used to assess the severity and prognosis of anti-NMDAR receptor encephalitis.3.Consciousness disorder,mental and behavioral abnormalities,the incidence of multiple clinical syndromes(more than 4)within short period(less than 2 weeks)and the high peak mRS scores are risk factors of poor efficacy of first-line immunotherapy,so rituximab is recommended to be co-administered with as early as possible.4.Patients who poorly respond to for first-line therapy should be treated with rituximab as early as possible,to achieve better results and reduce recurrence.5.Rituximab has significant damage to B lymphocytes and should be monitored periodically. |
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