| Background and ObjectiveSince anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis was discovered in 2007,autoimmune encephalitis is becoming an important and common encephalitis,especially for anti-neuronal cell surface or synaptic protein antibody-associated autoimmune encephalitis.However,anti-NMDAR encephalitis and anti-leucine-rich glioma-inactivated 1(LGI1)encephalitis seem to constitute most of the clinical cases in the published literature.Therefore,this study collected and analyzed the clinical characteristics and prognosis of patients with anti-NMDAR encephalitis and anti-LGI1 encephalitis in our hospital,and compared the two kinds of encephalitis,in order to help people improve the understanding of the two kinds of autoimmune encephalitis and provide reference for early diagnosis,differentiation,treatment and prognosis evaluation of the two kinds of encephalitis.Materials and MethodsThe clinical data of patients with anti-NMDAR encephalitis and anti-LGI encephalitis diagnosed in the First Affiliated Hospital of Zhengzhou University from January 2017 to October 2020 were analyzed retrospectively,including sex,age,prodromal symptoms,first symptoms,main symptoms,auxiliary examination(detection of autoantibodies in serum and cerebrospinal fluid,routine biochemistry of cerebrospinal fluid,head magnetic resonance imaging(MRI),PET-CT,CT or color ultrasoundchest of abdominal and pelvic,etc.),whether to stay in ICU,whether complicated with tumor,immunotherapy,length of stay,etc.And follow-up by telephone to know the prognosis.The modified Rankin scale(modified Rankin scale,mRS)was used to score the patients in acute stage,discharge and follow-up.mRS score:0～2 as good prognosis,3～6 as poor prognosis.The differences in general data,clinical symptoms,auxiliary examination,treatment and prognosis between the two groups were compared and analyzed,so as to provide a basis for accurate diagnosis,treatment and prognosis assessment of the two diseases.Results1.31 patients with anti-LGI1 encephalitis and 49 patients with anti-NMDAR encephalitis were included in this study.The median age and quartile interval of the former was 63(54-67)years,which was significantly higher than that of 25(19-34)years old of the latter(P=0.000).Male accounted for 71.0%in the former was higher than 49%of the latter,but there was no significant gender difference between the two types of encephalitis(P=0.053).The course median and quartile interval of the former was 56(30-60)days,which was significantly longer than that of the latter 15(7-26.5)days(P=0.000).The incidence of non-specific prodromal symptoms in the former was 9.7%,which was significantly lower than the 53.1%in the latter(P=0.000).The proportion of the former with memory impairment as the first symptom was 22.6%,which was significantly higher than 4.1%of the latter(P=0.029).The incidences of cognitive impairment,memory impairment and hyponatremia in the former were 74.2%,71.0%and 54.8%,respectively,which were significantly higher than those of the latter 52.1%(P=0.039),42.9%(P=0.014)and 2.0%(P=0.000).25.8%of patients with anti-LGI1 encephalitis had faciobrachial dystonic seizures,which was unique to this type of encephalitis,and the incidence of status epilepticus of patients with anti-LGI1 encephalitis was 6.5%,which was significantly lower than that of 28.6%in patients with anti-NMDAR encephalitis(P=0.016).At the same time,in the former,the median and quartile interval of hospital stay was 16(12-24)days,which were significantly lower than 23(17-29)days(P=0.006)in the latter.And the median and quartile interval of acute mRS score was 3(2-3)in the former,which was significantly lower than 4(3-5)(P=0.001)in the latter.The ICU occupancy rate of the former was 9.7%,which was significantly lower than that of the latter(51.0%)(P=0.000).2.The abnormal incidences of elevated cerebrospinal fluid pressure,elevated white blood cell count and elevated lymphocyte proportion in patients with anti-LGI1 encephalitis were 9.7%,12.9%and 32.3%,respectively,which were significantly lower than 40.8%(P=0.003),75.5%(P=0.000)and 81.6%(P=0.000)in patients with anti-NMDAR encephalitis.The incidence of chloride reduction in cerebrospinal fluid was 29.0%in patients with anti-LGI1 encephalitis,which was significantly higher than 2.0%in patients with anti-NMDAR encephalitis(P=0.001).The proportion of frontal lobe involvement of MRI in patients with anti-LGI1 encephalitis was 6.5%(2/31),which was significantly lower than 29.8%(14/47)in patients with anti-NMDAR encephalitis(P=0.013).Anti-LGI1 encephalitis often involved the hippocampus and basal ganglia,while the range of head MRI involvement in patients with anti-NMDAR encephalitis was wider.3.30 patients with anti-LGI1 encephalitis and 46 patients with anti-NMDAR encephalitis received first-line immunotherapy.The former was most commonly treated with glucocorticoid alone,accounting for 60.0%,which was significantly higher than that of the latter 30.4%(P=0.011),while the latter was most commonly treated with combined glucocorticoid and immunoglobulin,accounting for 47.8%.And the treatment of the later is more diversified.The proportion of patients with anti-LGI1 encephalitis treated with one first-line immunotherapy was 66.7%,which was significantly higher than 39.1%of patients with anti-NMDAR encephalitis(P=0.019),while the ratio of combined use of two first-line immunotherapy of anti-LGI1 encephalitis was 33.3%,significantly lower than 56.5%(P=0.048)of patients with anti-NMDAR encephalitis.The patients with two kinds of encephalitis responded well to immunotherapy,and there was no significant difference in mRS score at discharge(P=0.446).4.26 patients with anti-LGI1 encephalitis and 41 patients with anti-NMDAR encephalitis were followed up.The follow-up time median and quartile interval of patients with anti-LGI1 encephalitis was 24(12-39.5)months,while 26(16-35.5)months for patients with anti-NMDAR.There was no significant difference in follow-up time(P=0.990).The median and quartile interval of mRS score of the former was 1(0-2.25),which was significantly higher than 0(0-0)of the latter(P=0.005),and in the former,the percentage of patients who completely returned to normal was 46.2%,which was significantly lower than that of the latter(80.5%)(P=0.004).The proportion of sequelae and the proportion of sequelae of memory impairment in patients with anti-LGI1 encephalitis were 38.5%and 34.6%,which were significantly higher than 12.2%(P=0.012)and 7.3%(P=0.012)of patients with anti-NMDAR encephalitis.There was no significant difference in mortality between the two kinds of encephalitis(P=0.521).Conclusion1.Anti-LGI1 encephalitis is more common in elderly and male patients,with fewer prodromal symptoms,longer course of disease,lighter symptoms in acute stage,but more likely to leave sequelae of memory impairment.In contrast,the patients with anti-NMDAR encephalitis have younger age of onset,obvious prodromal symptoms,wide range of lesions,severe condition in acute stage,and are more likely to develop status epilepticus,but the long-term prognosis is relatively good.2.Anti-LGI1 encephalitis often involves the hippocampus and basal ganglia.Attacks of faciobrachial dystonic seizures and hyponatremia may be specific clinical manifestations of the disease. |
PDF Full Text Request