Clinical Analysis Of Anti-leucine-rich Glioma Inactivated 1 Protein Antibody Associated Limbic Encephalitis

Object: This study was to describe the clinical features of Anti-leucine-rich glioma inactivated protein 1(LGI1)antibody associated limbic encephalitis for a further understanding of this disease.Methods: Clinical data including clinical manifestation,laboratory and radiological findings,prognosis of 22 patients diagnosed by People’s Hospital of Zhengzhou University from March 2015 to January 2018 were analyzed.Results: This case series included 22 patients with 15 males and 7 females aging from 31 to 78 years-old.The median age was 53 years.Cognitive dysfunction occurred in 19 patients(86%),seizuresin 14(64%),psychosisin 12(86%),and faciobrachial dystonic seizure in 10(45%),refractory hyponatremia in 6 cases(27%),autonomic dysfunction in 5 cases(23%).Sleep disorder was shown in 9 cases.1 case only presented typically acute Guillain-Barre syndrome(GBS)without any other symptoms.12 cases(55%)had mild abnormalities in routine examination of cerebrospinal fluid.All patients were positive for anti-LGI1 antibodies in serum samples and 14 were positive in cerebrospinal fluid.14 cases(64%)had MRI abnormolity,of which 13 showed unilateral or bilateral abnormalities of the temporal lobe.8/17 cases(47%)had EEG abnormality.Only 1 case had elevated tumor markers.The time interval from the appearance of clinical symptoms to diagnosis ranged from 4 days to 2 years,the median was 30 days.19 patients received immunotherapy,3 patients rejected and only received symptomatic treatment.The median m RS score was 3(range: 2-4)at disease onset,2(range: 0-2)after treatment,the difference was statistically significant(Z=-4.208,P=0.000).During a 2-29 months follow-up,3 patients were lost.At the final follow-up,the median mRS score was 1(range:0-2).15 patients were basically or completely normal(m RS score 0-1 point).The remaining symptoms were mainly memory loss,sleep disorders,inattention,personality changes.During the follow-up period,no relapse symptoms was observed.Conclusion: The most comman clinical manifestations of LGI 1 antibody associated encephalitis were cognitive dysfunction,and epileptic seizures,psychosis and FBDS.Uncommonly,it may also manifest as episodic symptoms such as vertical hair,dizziness,amaurosis.The seropositivity was higher than that of the cerebrospinal fluid with indirect immunofluorescence.Mesiotemporal abnormal was the common pattern of MRI changes.Prognosis was usually favourable.Tumor was uncommon.After immunotherapy,some patients can return to normal completely.The remaining symptoms were mainly memory loss,inattention,personalit changes.Relapse was rare.