Heterogeneity of clinical features, EEG and brain imaging findings in anti‑leucine‑rich glioma‑inactivated protein 1 autoimmune encephalitis: a retrospective case series study and review of the literature  

作　　者：Emily Yixuan Huang Hongfeng Gao Ning Zhong 

机构地区：[1]Department of Molecular and Cell Biology,University of California,Berkeley,CA 94720,USA [2]Department of Molecular and Cell Biology and Helen Wills Neuroscience Institute,University of California,Berkeley,CA 94720,USA [3]Department of Neurology,Kaiser Permanente Sacramento Medical Center,Sacramento,CA 95825,USA [4]North Valley Regional Epilepsy Center,Kaiser Permanente Sacramento Medical Center,Sacramento,CA 95825,USA

出　　处：《Acta Epileptologica》2023年第3期173-184,共12页癫痫学报（英文）

摘　　要：Background Anti-leucine-rich glioma-inactivated 1(LGI-1)autoimmune encephalitis(AE),characterized by rapid decline of memory,seizures,and neuropsychiatric abnormalities,is a rare but devastating disorder.Early diagnosis and treatment are essential to prevent long-term sequelae.In this report,we provide a detailed description of clinical characteristics,laboratory test results,imaging,and electroencephalography(EEG)findings,as well as treatment responses of eight patients with anti-LGI-1 AE treated at our center.Case presentation At the onset,all eight patients presented with confusion/memory deterioration,seizures(including faciobrachial dystonic seizures or other types of seizure),and behavioral changes such as hallucination,paranoia,and anxiety.Four patients were found with severe hyponatremia.Anti-LGI1 antibodies were detected in the cerebrospinal fluid and/or serum of all patients.For patients with faciobrachial dystonic seizures,no discernible scalp EEG change was detected,while EEG recording of patients experiencing other types of seizure showed focal slowing,focal epileptiform discharges,and focal onset seizures.All patients showed abnormal brain magnetic resonance imaging signals,mainly involving the mesial temporal lobe and the hippocampus.In addition,one patient also experienced fulminant cerebral edema during the acute phase of the illness.All patients received immunotherapy and anti-seizure medications and achieved good seizure control.Nevertheless,these patients continued to experience cognitive impairment during their long-term follow-ups.Conclusions The care of anti-LGI1 AE patients requires rapid evaluation,prompt initiation of immunotherapy,and long-term follow-up.The long-term presence of neurocognitive complications observed in these patients underline the importance of developing reliable biomarkers that can distinguish between different subtypes of this disease with heterogeneous clinico-electrographico-radiological features.Further research is needed to understand the molecular mechanisms un

关 键 词：Anti-leucine-rich glioma-inactivated 1 Autoimmune encephalitis Faciobrachial dystonic seizures Epilepsy 

分 类 号：R742.9[医药卫生—神经病学与精神病学] R740[医药卫生—临床医学]