title: VGKC complex antibodies in pediatric severe acute encephalitis: a study and literature review.
abstract: BACKGROUND
  Antibodies to surface proteins like voltage-gated potassium channel (VGKC) complexes are increasingly found in different neurologic diseases and encephalitis in adults and recently, in children. Detecting such antibodies can help identify forms of encephalitis that may respond to immuno-therapies. However, there are few reports on VGKC complex antibodies in pediatric severe acute encephalitis.
  
  
  METHODS
  This study retrospectively reviewed antibodies to VGKC, leucine-rich glioma-inactivated 1 (Lgi1), and contactin-associated protein-like 2 (Caspr2) in 46 children with severe acute encephalitis. Published cases of VGKC complex antibodies in pediatric encephalitis in the period of 2000-2012 were also reviewed.
  
  
  RESULTS
  Elevated VGKC complex antibodies (>100pM) were detected in one of the 46 children with severe acute encephalitis. The 4-year and 6-month-old girl presented with seizure and disturbed consciousness. Viral PCR/culture and serologic evidence of influenza A infection was noted. She also had complications of epilepsy, impaired cognition, and altered behavior and psychology. Antibodies to Lgi1 and Caspr2 were not detected. Ten previously published reports revealed that VGKC complex antibodies can occur in children with limbic encephalitis and acute or sub-acute encephalitis.
  
  
  CONCLUSIONS
  The incidence of VGKC complex antibodies in pediatric severe acute encephalitis is not high with only one (2.2%) of 46 children in this study. And, this is the first report on the association of VGKC complex antibodies and patients with influenza A-related severe acute encephalitis. The mechanism of VGKC complex antibodies in pediatric severe acute encephalitis warrants further study.
authors.affiliation: Division of Pediatric Neurology, College of Medicine, Chang Gung University, Taoyuan, Taiwan.
authors.forename: Jainn-Jim
authors.initials: JJ
authors.lastname: Lin
authors.forename: Kuang-Lin
authors.initials: KL
authors.lastname: Lin
authors.forename: Shao-Hsuan
authors.initials: SH
authors.lastname: Hsia
authors.forename: Huei-Shyong
authors.initials: HS
authors.lastname: Wang
authors.forename: Cheng-Hsun
authors.initials: CH
authors.lastname: Chiu
authors.lastname: CHEESE Study Group
mesh_terms: D000293:Adolescent; D001323:Autoantibodies / Q000097:blood*; D001324:Autoantigens / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D004660:Encephalitis / Q000097:blood / Q000276:immunology*; D005260:Female; D006801:Humans; D007223:Infant; D009980:Influenza A virus; D007251:Influenza, Human / Q000150:complications / Q000276:immunology; D008297:Male; D024642:Potassium Channels, Voltage-Gated / Q000276:immunology*; D012189:Retrospective Studies
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D024642:Potassium Channels, Voltage-Gated