Shekeeb S Mohammad1, Kate Sinclair, Sekhar Pillai, Vera Merheb, Tim D Aumann, Deepak Gill, Russell C Dale, Fabienne Brilot. 1. 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.
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.
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: Thaís Armangue; Germán Moris; Verónica Cantarín-Extremera; Carlos Enrique Conde; Kevin Rostasy; Maria Elena Erro; Juan Carlos Portilla-Cuenca; Eulàlia Turón-Viñas; Ignacio Málaga; Beatriz Muñoz-Cabello; Carmen Torres-Torres; Sara Llufriu; Luis González-Gutiérrez-Solana; Guillermo González; Ignacio Casado-Naranjo; Myrna Rosenfeld; Francesc Graus; Josep Dalmau Journal: Neurology Date: 2015-10-21 Impact factor: 9.910