title: Herpes simplex encephalitis relapse with chorea is associated with autoantibodies to N-Methyl-D-aspartate receptor or dopamine-2 receptor.
abstract: BACKGROUND
  Movement disorder relapses after herpes simplex virus 1 (HSV1) encephalitis have been hypothesized to be secondary to postviral autoimmunity. Recently, a proportion of patients with HSV1 encephalitis (HSE) were shown to produce autoantibodies against N-methyl-D-aspartate receptor (NMDAR).
  
  
  METHODS
  We measured autoantibodies against NMDAR and dopamine-2 receptor (D2R) expressed at the cell surface in the stored acute serum of 9 children with HSE, 3 of whom had a relapsing course with chorea.
  
  
  RESULTS
  The 3 patients with chorea had elevated autoantibodies against NMDAR (n = 1), D2R (n = 1), or both (n = 1), whereas patients without chorea were negative (n = 6). The prospectively identified patient with chorea and NMDAR autoantibodies improved after early treatment with steroids, intravenous immunoglobulin, and cyclophosphamide, with reduction in serum NMDAR antibody titers.
  
  
  CONCLUSIONS
  These autoantibody findings lend support to the autoimmune hypothesis and the early use of immune suppression in post-HSE chorea.
authors.affiliation: Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Kids Research Institute at the Children's Hospital at Westmead, University of Sydney, Westmead, Australia; Department of Neurosciences, Royal Children's Hospital, Brisbane, Australia; School of Medicine, University of Queensland, Brisbane, Australia; TY Nelson Department of Neurology and Neurosurgery, Children's Hospital at Westmead, Westmead, Australia.
authors.forename: Shekeeb S
authors.initials: SS
authors.lastname: Mohammad
authors.forename: Kate
authors.initials: K
authors.lastname: Sinclair
authors.forename: Sekhar
authors.initials: S
authors.lastname: Pillai
authors.forename: Vera
authors.initials: V
authors.lastname: Merheb
authors.forename: Tim D
authors.initials: TD
authors.lastname: Aumann
authors.forename: Deepak
authors.initials: D
authors.lastname: Gill
authors.forename: Russell C
authors.initials: RC
authors.lastname: Dale
authors.forename: Fabienne
authors.initials: F
authors.lastname: Brilot
mesh_terms: D001323:Autoantibodies / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D002819:Chorea / Q000097:blood / Q000150:complications / Q000276:immunology*; D020803:Encephalitis, Herpes Simplex / Q000097:blood / Q000150:complications / Q000276:immunology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D017448:Receptors, Dopamine D2 / Q000276:immunology*; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology*; D012008:Recurrence
chemical_list: D001323:Autoantibodies; D017448:Receptors, Dopamine D2; D016194:Receptors, N-Methyl-D-Aspartate
keywords: NMDA receptor; autoantibody; chorea; dopamine-2 receptor; herpes simplex encephalitis