| Background and ObjectivesAutoimmune encephalitis(AE)is a kind of encephalitis syndrome which has abnormal immune response to the central nervous system due to the presence of positive antibodies against autoantigen in serum and / or cerebrospinal fluid.Usually acute or subacute onset(? 3 months).The common clinical manifestations are mental and behavioral abnormalities,cognitive impairment,near memory loss,seizures,speech disorders,involuntary movements,decreased consciousness or coma,autonomic nervous dysfunction and so on.The main diagnostic method of autoimmune encephalitis is the presence of related anti-neuron antibodies in blood and cerebrospinal fluid.The detection of specific autoantibodies determines the diagnosis and immunological subtypes of autoimmune encephalitis,each subtype has its own clinical characteristics,imaging examination and tumor complication rate are also different,and is helpful for the differential diagnosis of atypical clinical cases.Most of the different types of autoantibodies correspond to specific neurological symptoms and have strong specificity and directivity in the diagnosis of etiology.In the past 10 years,due to the development and popularization of laboratory antibody detection technology,different types of antibody positive AE have been diagnosed almost every year,and the anti-neuron antibody spectrum has been greatly expanded,but most of them are reported as autoimmune encephalitis with single anti-neuronantibody positive.In recent years,it has been found that two or more positive antibodies can be detected in the same patient,which is relatively rare in AE.There are only a few related reports in the relevant literature,and its specific mechanism and clinical significance are not clear.In this study,we collected cases of autoimmune encephalitis with two or more positive anti-neuron antibodies diagnosed in the first affiliated Hospital of Zhengzhou University from January 2015 to May 2019.we used the method of descriptive study to summarize the clinical characteristics of these cases from four aspects: clinical manifestation,imaging examination,antibody detection results,treatment and prognosis,and analyze and discuss their clinical significance,in order to improve the clinical understanding of this kind of disease.Materials and MethodsA total of 255 patients with autoimmune encephalitis were collected from the first affiliated Hospital of Zhengzhou University from January 2015 to May 2019.7patients with positive anti-neuron antibodies were further screened as the subjects of this study.The clinical data of all patients were analyzed and summarized by descriptive study,including clinical symptoms,antibody detection and imaging results,clinical treatment and prognosis.Among them,the autoimmune encephalitis antibody was detected simultaneously in blood and cerebrospinal fluid by indirect immunofluorescence(the initial dilution titer was 1:10 and 1:1 respectively).The detection mainly included routine autoimmune encephalitis antibodies(anti-LGI1,NMDAR,GABABR,AMPA1/2 and CASPR2 antibodies)and paraneoplastic related antibodies(anti-Ma2,anti-Amphiphysin,anti-Yo,anti-Hu,anti-CV2,anti-Ri).At the same time,the comprehensive tumor screening of patients was collected to determine whether it was complicated with tumor or not.All patients were followed up by telephone in May 2019.The modified Rankin score was used to evaluate the clinical prognosis,and the score ? 2 was defined as good recovery of neurological function.Recurrence refers to the recurrence of neurological symptoms after more than 2months of stable or recovery of symptoms,or aggravation of symptoms on theoriginal basis(m RS score increased by 1 point or more).Results1.Among the 255 patients diagnosed with autoimmune encephalitis,there were only 7 cases with positive anti-neuron antibodies at the same time,accounting for2.7%,which is rare in clinic.Of the 7 patients,5 were female(5/7)and 2 male(2/7),with a male to female ratio of 1:2.5.The median age of onset was 60 years old(between 43 and 84 years old).2.All patients had acute or subacute onset(3 days to 3 months),and their clinical manifestations and imaging features were complex and changeable.The tumor complication rate was relatively increased.Among the 7 patients,2 cases were confirmed to be complicated with tumor(2/7),and 2 cases were highly suspected to be complicated with tumor(2/7).Among them,1 patient was complicated with uterine leiomyoma,and there are no reports to determine whether the tumor is related to autoimmune antibodies.3.The antibody results showed that anti-NMDAR encephalitis combined with other antibodies accounted for 57%,followed by anti-GABABR(29%)and anti-LGI1encephalitis(14%).And 71% of the patients were associated with paraneoplastic related antibodies(5/7).The specific results were as follows: 4 patients with anti-NMDAR encephalitis were complicated with anti-LGI1 antibody(2/4),anti-Yo antibody(1/4)and anti-Ma2 antibody(1/4),2 patients with anti-GABABR encephalitis were positive for anti-Amphiphsin and anti-Hu antibodies,and 1 patient with anti-LGI1 encephalitis was positive for anti-Hu antibody.4.Among all the patients,6 cases were given first-line immunotherapy,only 1case was treated with symptomatic treatment,and the symptoms were improved after treatment(7/7).There were 3 cases of recurrence(3/7).By the end of follow-up,neurological function recovered well in 5 cases(5/7)and died in 2 cases(2/7).Conclusion1.The incidence of autoimmune encephalitis with multiple anti-neuronantibodies is low.2.The superposition of antibody types may lead to the superposition of clinical syndromes.Atypical symptoms can easily lead to misdiagnosis and missed diagnosis,and may be prone to recurrence and poor prognosis.3.The existence of two kinds of anti-neuron antibodies may make the range of potential tumors wider,increase the possibility of potential malignant tumors,and may predict specific malignant tumors. |
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