Clinical Study Of Autoimmune Encephalitis

Background and Objective:Autoimmune encephalitis(AE)refers to a class of diseases that are mediated by the autoimmune mechanism against the central nervous system and cause neurological deterioration.The symptoms of AE include seizure,memory dysfunction,abnormal behavior,decreased consciousness and so on.Since the pioneering discovery of N-methyl-d-aspartate receptor(NMDAR)autoantibodies,autoimmune encephalitis associated with autoantibodies to synaptic proteins of neurons is increasingly recognized.However,it is a difficult clinical diagnosis due to the similarities in the clinical,imaging and laboratory findings of many forms of diseases.Early diagnosis and timely immune treatment can improve the clinical symptoms.To improve the recognition of AE,the clinical features,treatment and prognosis were analyzed in X X Hospital.Methods:We analysis the clinical presentation,magnetic resonance imaging(MRI),cerebrospinal fluid(CSF),electroencephalograms(EEG)and treatment outcome of 15 cases with autoimmune encephalitis.Result:1.Among 6 cases with anti-NMDAR encephalitis,50% patients were male.Theaverage age was 30 years old.The epileptic attack as first symptom was observed in 3patients.The mental and behavior disorder as first symptom was observed in 3 patients.5patients showed abnormal movements.The MRI showed diffusion weighted imaging(DWI)high signal intensity in bilateral thalamic regions in one patient.In another patient,the MRI showed T2/FLAIR high signal intensity in left temporal region,anti-NMDAR encephalitis of the left 4 patients showed no changes in the MRI.The CSF findings were in most respects similar to viral encephalitis.2 cases had teratoma,one case had small cell lung carcinoma,and the symptoms were recovered partially after treatment of immunoagents.2.Among 5 patients with anti-?-aminobutyric acid(GABA)B receptor encephalitis,4 patients were male.The average age was 61 years old.The clinical features of these patients included seizures(5/5),psychiatric symptoms(5/5),cognitive disorders(5/5).CSF and serum anti–GABA(B)receptor antibodies were positive in all patients.Electroencephalograms revealed epileptic discharges in 1 patient.Brain MRI showed abnormal signal in 2 patients,located in the hippocampus,temporal lobes regions.Small cell lung cancer was found in 1 patient,ribs epithelioid hemangioendothelioma was found in another patient.All patients received immunotherapy.3 patients showed complete or partial neurological relief to immunotherapy,while the other 2 patients,one with lung cancer died due to the neoplastic complication,one with ribs epithelioid hemangioendothelioma,who had a poor prognosis.3.Among 4 patients with Anti-leucine-rich glioma-inactivated 1 antibody encephalitis(LGI1),mean age 56 years(range 36-70),3 patients were male.1 patient presented with faciobrachial dystonic seizure onset,another with pain,the others with hypomnesis.Among the 4 patients,2 patients experienced seizures,while 4 patients showed cognitive dysfunction,mainly deficits in memory.1 patient exhibited hallucination,1 patient had insomnia,1 patient with emotional instability.No tumors were detected in any patient.2 patients had hyponatremia.Every patient was examined by MRI,The MRI showed T2 high signal intensity in bilateral temporal lobe of one patient.1patient underwent positron emission tomography(PET)showed no systemic tumors andmatabolic abnormalities.Patients with seizures responded poorly to antiepileptic drugs.Conclusion:1.The first symptom of anti-NMDAR encephalitis often was psychiatric or seizures.Abnormal movements and autonomic instability can be seen in these patients.The EEG and MRI findings in some patients were no specificity.Patients associated with ovarian tumors underwent tumor excision and immunotherapy had remarkable efficacy.2.The mainly clinical manifestations in patients with anti-GABA(B)receptor encephalitis were seizures,psychiatric and cognitive disorder.Most patients with prominent epilepsy usually refractory to multiple anti-epileptic agents and shows response to immunotherapy.Patients with underlying tumor have a relatively poor prognosis.3.Anti-leucine-rich glioma-inactivated 1 antibody encephalitis(LGI1)is characterized by faciobrachial dystonic seizures(FBDS),memory loss,hyponatremia and antibodies against the leucine-rich.There is some evidence to suggest that early treatment of LGI-1antibody encephalitis may be associated with a better functional.