Herpes simplex encephalitis relapse with chorea is associated with autoantibodies to N-Methyl-D-aspartate receptor or
Shekeeb S Mohammad1, Kate Sinclair, Sekhar Pillai
1Neuroimmunology 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.
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.
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