Herpes simplex encephalitis relapse with chorea is associated with autoantibodies to N-Methyl-D-aspartate receptor or dopamine-2 receptor

Mov Disord. 2014 Jan;29(1):117-22. doi: 10.1002/mds.25623. Epub 2013 Oct 1.


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.

Keywords: NMDA receptor; autoantibody; chorea; dopamine-2 receptor; herpes simplex encephalitis.

MeSH terms

  • Autoantibodies / blood*
  • Child
  • Child, Preschool
  • Chorea / blood
  • Chorea / complications
  • Chorea / immunology*
  • Encephalitis, Herpes Simplex / blood
  • Encephalitis, Herpes Simplex / complications
  • Encephalitis, Herpes Simplex / immunology*
  • Female
  • Humans
  • Infant
  • Male
  • Receptors, Dopamine D2 / immunology*
  • Receptors, N-Methyl-D-Aspartate / immunology*
  • Recurrence


  • Autoantibodies
  • Receptors, Dopamine D2
  • Receptors, N-Methyl-D-Aspartate