Clinical Features Of 22 Cases Of Autoimmune Epilepsy

Objective: A retrospective analysis of the seizure type,seizure semiologies,EEG findings and treatment prognosis of patients with autoimmune epilepsy(AE)was conducted to help clinicians better understand the clinical characteristics of the disease,and achieve clinical diagnosis and treatment early.Methods:1 we collected serum and/or CSF anti-neuron antibody(Ab)-positive patients in the second hospital of Hebei medical university from January 2018 to December 2019 by searching electronic medical records system.The demographic characteristics such as age,sex,etc.and clinical characteristics of patients with seizures included seizure type,frequency and duration of each seizure,and clinical manifestations,age of seizure onset,time interval from onset to diagnosis,laboratory examination,cerebrospinal fluid analysis,antibody type,EEG and imaging manifestations,and immunosuppressive and AEDs,etc were collected for retrospective analysis.2 The efficacy of immunosuppressive and AEDs was assessed by following up.A favorable response to immunotherapy was defined as > 50% reduction in seizure frequency.Results:We identified a total of 30 patients with autoimmune encephalitis.Among them,22 patients were with seizures,for whom 5 patients with NMDA-R Ab,5 with LGI1 Ab,6 with GABAB-R Ab,6 with GAD65 Ab.Of the patients with LGI1 Ab,2 with FBDS.These 22 patients presented with various forms of seizures,including FAS,FIAS,GTCS,and so on.The main manifestations of GAD65-Ab and LGI1 Ab encephalitis was temporal lobe epilepsy.One characteristic phenotype described in the two patients with LGI1 was FBDS,which were brief focal dystonic motor seizures occurring multiple times a day.They have a characteristic stereotypic contraction of the face,arm,and legin.While those with GABAB-R Ab were mainly characterized by frequent GTCS.Patients with GABAB-R Ab presented mainly with extensive encephalopathy such as psychiatric symptoms,behavioral abnormalities and multiple kind of seizures.Each patient can have one or more forms of seizures or seizure evolved from one to another and seizures are very frequent and have a short duration.Among the 22 patients with epileptic seizures,epileptic discharge in EEG for most patients was mainly presented temporal lobe onset,extratemporal discharge can also be found in several patients;However,most patients showed focal or extensive slow wave,and a few showed normal EEG.Four patients had seizures as their only presentation without symptoms of limbic encephalitis,three of whom were with GAD65 Ab.Nearly half of the patients had CSF findings consistent with inflammation and 45% had medial temporal lobe involvement in MRI.Most of the patients were treated with immunosuppresive and AEDs therapy.The seizure frequency of 77.27% patients was reduced by 50% and 68.18% patients had complete seizure freedom during following up.This study focused on the clinical features of 6 patients with GAD65 Ab and DM1,of whom both serum and CSF Ab were positive.All 6 patients received immunotherapy,with or without AEDs,and achieved significant improvement in seizure frequency or achieved seizure freedom.Conclusion:1 Patients with autoimmune encephalitis in this study had a high rate of seizures,accounting for 73%.Some encephalitis has a special form of attack: the main manifestations of GAD65-Ab and LGI1 Ab encephalitis were TLE,in which FBDS was the characteristic manifestation of LGI1 Ab encephalitis;Patients with GABAB-R Ab encephalitis mainly manifested in frequent GTCS and patients with NMDA-R Ab encephalitis Manifested mainly with generalized encephalopathy such as seizures,mental symptoms,behavioral abnormalities.2 The seizures of AE were characterized by very frequent seizures and a short duration.Each patient may have one or more seizures.3 The imaging findings of patients with AE were more frequent in the medial temporal lobe involvement.The EEG of most patients showed nonspecific background abnormalities,and the intermittent discharge was mainly in temporal lobe onset.4 Most patients with AE had a favorable response to immunotherapy combined with AEDs.