title: Clinical characterization of autoimmune LGI1 antibody limbic encephalitis.
abstract: OBJECTIVE
  Autoimmune encephalitis associated with antibodies to leucine-rich glioma inactivated 1 (LGI1) has recently been identified and is characterized by an acute to subacute onset of cognitive impairment and convulsion, faciobrachial dystonic seizures (FBDSs), and psychiatric disturbances. This study analyzed the clinical characteristics and outcomes of 10 patients with LGI1 antibody encephalitis in order to further understand this disease and to improve its therapeutic strategies.
  
  
  METHODS
  Between January 2013 and March 2015, we identified 10 patients with LGI1 antibody encephalitis. We retrospectively analyzed the clinical details, laboratory results, electrophysiological and imaging findings, and the treatment outcomes.
  
  
  RESULTS
  All patients tested had LGI1 antibodies. Immunotherapy was effective in all patients. Seizures in patients with FBDS showed a poor response to antiepileptic drugs. Two patients examined by magnetoencephalogram (MEG) during the acute disease phase showed a small quantity of spike-wave dipoles in the temporal lobe close to the lateral fissure and insular lobe.
  
  
  CONCLUSIONS
  Patients with LGI1 antibody encephalitis responded well to immunotherapy. We speculate that FBDS is likely a form of insular epilepsy.
authors.affiliation: Department of Neurology, Xuanwu Hospital, Capital Medical University, The Beijing Key Laboratory of Neuromodulation, Beijing, China.
authors.forename: Lehong
authors.initials: L
authors.lastname: Gao
authors.affiliation: Department of Neurology, Xuanwu Hospital, Capital Medical University, The Beijing Key Laboratory of Neuromodulation, Beijing, China.
authors.forename: Aihua
authors.initials: A
authors.lastname: Liu
authors.affiliation: Department of Neurology, Xuanwu Hospital, Capital Medical University, The Beijing Key Laboratory of Neuromodulation, Beijing, China.
authors.forename: Shuqin
authors.initials: S
authors.lastname: Zhan
authors.affiliation: Department of Neurology, Xuanwu Hospital, Capital Medical University, The Beijing Key Laboratory of Neuromodulation, Beijing, China.
authors.forename: Li
authors.initials: L
authors.lastname: Wang
authors.affiliation: Department of Neurology, Xuanwu Hospital, Capital Medical University, The Beijing Key Laboratory of Neuromodulation, Beijing, China.
authors.forename: Liping
authors.initials: L
authors.lastname: Li
authors.affiliation: Department of Nuclear Medicine, Xuanwu Hospital, Capital Medical University, Beijing, China.
authors.forename: Le
authors.initials: L
authors.lastname: Guan
authors.affiliation: Department of Neurology, Xuanwu Hospital, Capital Medical University, The Beijing Key Laboratory of Neuromodulation, Beijing, China.
authors.forename: Xin
authors.initials: X
authors.lastname: Zhao
authors.affiliation: Department of Neurology, Xuanwu Hospital, Capital Medical University, The Beijing Key Laboratory of Neuromodulation, Beijing, China.
authors.forename: Xiating
authors.initials: X
authors.lastname: Zhang
authors.affiliation: Department of Neurology, Xuanwu Hospital, Capital Medical University, The Beijing Key Laboratory of Neuromodulation, Beijing, China. Electronic address: doctorwangyuping@163.com.
authors.forename: Yuping
authors.initials: Y
authors.lastname: Wang
mesh_terms: D000328:Adult; D000368:Aged; D000927:Anticonvulsants / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000097:blood* / Q000175:diagnosis* / Q000628:therapy; D015415:Biomarkers / Q000097:blood; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007167:Immunotherapy / Q000639:trends; D047908:Intracellular Signaling Peptides and Proteins; D020363:Limbic Encephalitis / Q000097:blood* / Q000175:diagnosis* / Q000628:therapy; D015225:Magnetoencephalography / Q000379:methods; D008297:Male; D008875:Middle Aged; D011506:Proteins / Q000378:metabolism*; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D000927:Anticonvulsants; D001323:Autoantibodies; D015415:Biomarkers; D047908:Intracellular Signaling Peptides and Proteins; C116499:LGI1 protein, human; D011506:Proteins
keywords: Autoimmune encephalitis; LGI1; Limbic encephalitis