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

Authors

  • Shekeeb S. Mohammad MBBS,

    1. Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Kids Research Institute at the Children's Hospital at Westmead, University of Sydney, Westmead, Australia
    2. Department of Neurosciences, Royal Children's Hospital, Brisbane, Australia
    3. School of Medicine, University of Queensland, Brisbane, Australia
    4. TY Nelson Department of Neurology and Neurosurgery, Children's Hospital at Westmead, Westmead, Australia
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  • Kate Sinclair FRACP,

    1. Department of Neurosciences, Royal Children's Hospital, Brisbane, Australia
    2. School of Medicine, University of Queensland, Brisbane, Australia
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  • Sekhar Pillai FRACP,

    1. Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Kids Research Institute at the Children's Hospital at Westmead, University of Sydney, Westmead, Australia
    2. TY Nelson Department of Neurology and Neurosurgery, Children's Hospital at Westmead, Westmead, Australia
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  • Vera Merheb BSc,

    1. Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Kids Research Institute at the Children's Hospital at Westmead, University of Sydney, Westmead, Australia
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  • Tim D. Aumann PhD,

    1. Florey Institute of Neuroscience and Mental Health, University of Melbourne, Parkville, Australia
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  • Deepak Gill FRACP,

    1. TY Nelson Department of Neurology and Neurosurgery, Children's Hospital at Westmead, Westmead, Australia
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  • Russell C. Dale PhD,

    Corresponding author
    1. Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Kids Research Institute at the Children's Hospital at Westmead, University of Sydney, Westmead, Australia
    2. TY Nelson Department of Neurology and Neurosurgery, Children's Hospital at Westmead, Westmead, Australia
    • Correspondence to: Dr. Russell C. Dale, Clinical School, Children's Hospital at Westmead, Locked Bag 4001, Westmead, NSW 2145, Australia; Russell.dale@health.nsw.gov.au

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  • Fabienne Brilot PhD

    1. Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Kids Research Institute at the Children's Hospital at Westmead, University of Sydney, Westmead, Australia
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  • Relevant conflicts of interest/financial disclosures: A patent has been filed by FB and RCD (University of Sydney) claiming D2R as target for autoantibodies.

  • Full financial disclosures and author roles may be found in the online version of this article.

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. © 2013 International Parkinson and Movement Disorder Society

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