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

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

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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.



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).


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.


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.


These autoantibody findings lend support to the autoimmune hypothesis and the early use of immune suppression in post-HSE chorea.


NMDA receptor; autoantibody; chorea; dopamine-2 receptor; herpes simplex encephalitis

[Indexed for MEDLINE]

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