title: Limbic Encephalitis Associated with Anti-γ-aminobutyric Acid B Receptor Antibodies: A Case Series from China.
abstract: BACKGROUND
  Autoimmune encephalitis associated with antibodies against γ-aminobutyric acid B receptor (GABA B R) in patients with limbic encephalitis (LE) was first described in 2010. We present a series of Han Chinese patients for further clinical refinement.
  
  
  METHODS
  Serum and cerebrospinal fluid (CSF) samples from patients referred to the program of encephalitis and paraneoplastic syndrome of Peking Union Medical College Hospital were tested with indirect immunofluorescence. Clinical information of patients with anti-GABA B R antibody positivity was retrospectively reviewed, and descriptive statistical analysis was performed.
  
  
  RESULTS
  All eighteen anti-GABA B R antibody-positive cases had limbic syndromes, and electroencephalogram (EEG) or neuroimaging evidence fulfilled the diagnostic criteria of LE. Four patients had additional antibodies against Hu in serum and one had anti-N-methyl-d-aspartate receptor antibody in both sera and CSF. Seventeen (17/18) patients presented with new-onset refractory seizure or status epileptics. Twelve (12/18) patients had memory deficits, 11 (11/18) patients had personality change, 7 (7/18) patients had disturbance of consciousness, and 3 (3/18) patients showed cerebellar dysfunction. One patient with LE had progressive motor and sensory polyneuropathy. Lung cancer was detected in 6 (6/18) patients. Ten (10/18) patients showed abnormality in bilateral or unilateral mediotemporal region on magnetic resonance imaging. Ten (10/18) patients had temporal lobe epileptic activity with or without general slowing on EEG. Seventeen patients received immunotherapy and 15 of them showed neurological improvement. Four patients with lung cancer died within 1-12 months due to neoplastic complications.
  
  
  CONCLUSIONS
  Our study demonstrates that most Han Chinese patients with anti-GABA B R antibody-associated LE have prominent refractory epilepsy and show neurological improvement on immunotherapy. Patients with underlying lung tumor have a relatively poor prognosis. Testing for anti-GABA B R antibodies is necessary for patients with possible LE or new-onset epilepsy with unknown etiology.
authors.forename: Hong-Zhi
authors.initials: HZ
authors.lastname: Guan
authors.forename: Hai-Tao
authors.initials: HT
authors.lastname: Ren
authors.forename: Xun-Zhe
authors.initials: XZ
authors.lastname: Yang
authors.forename: Qiang
authors.initials: Q
authors.lastname: Lu
authors.forename: Bin
authors.initials: B
authors.lastname: Peng
authors.forename: Yi-Cheng
authors.initials: YC
authors.lastname: Zhu
authors.forename: Xiao-Qiu
authors.initials: XQ
authors.lastname: Shao
authors.forename: Yong-Qiang
authors.initials: YQ
authors.lastname: Hu
authors.forename: Dong
authors.initials: D
authors.lastname: Zhou
authors.affiliation: Department of Neurology, Peking Union Medical College Hospital; Neuroscience Center, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
authors.forename: Li-Ying
authors.initials: LY
authors.lastname: Cui
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000276:immunology*; D002681:China; D004569:Electroencephalography; D004827:Epilepsy / Q000276:immunology* / Q000473:pathology*; D005260:Female; D006801:Humans; D020363:Limbic Encephalitis / Q000276:immunology* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology*; D012189:Retrospective Studies; D005680:gamma-Aminobutyric Acid / Q000378:metabolism
chemical_list: D001323:Autoantibodies; D016194:Receptors, N-Methyl-D-Aspartate; D005680:gamma-Aminobutyric Acid