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Herpes simplex virus encephalitis (HSVE) is a devastating condition that relapses, often with a chorea in children, despite adequate antiviral treatment. At relapse, evidence of viral replication is frequently absent, suggesting that the relapse may be immune-mediated. Seven children who had a neurological relapse following their initial encephalitis, identified from 20 cases of pediatric HSVE, were studied. Serum and/or cerebrospinal fluid (CSF) were tested for N-methyl-D-aspartate receptor (NMDAR) and other antibodies previously reported in central nervous system autoimmunity. Five of the 7 relapsing children had choreoathetosis; 2 of these were NMDAR antibody-positive, 2 were negative (1 with HSV-positive CSF), and 1 was not available for testing. An additional patient, who relapsed with cognitive regression but with no movement disorder, was also NMDAR antibody-positive. In 2 of the NMDAR antibody-positive patients who were treated at relapse and in 1 who was treated only after 10 years of having a relapsing encephalopathy, a beneficial response was observed. Neurological relapses after HSVE may frequently be immune-mediated, particularly in children with chorea. NMDAR antibodies are common, and immunotherapy may be beneficial.

Original publication

DOI

10.1002/mds.25626

Type

Journal article

Journal

Mov Disord

Publication Date

01/2014

Volume

29

Pages

90 - 96

Keywords

N-methyl-D-aspartate (NMDA) receptor, choreoathetosis, encephalitis, herpes simplex virus, movement disorder, relapsing, Adolescent, Anti-N-Methyl-D-Aspartate Receptor Encephalitis, Autoantibodies, Child, Preschool, Encephalitis, Herpes Simplex, Female, Humans, Infant, Male, Receptors, N-Methyl-D-Aspartate, Recurrence