title: Viral triggering of anti-NMDA receptor encephalitis in a child - an important cause for disease relapse.
abstract: Herpes simplex encephalitis (HSE) in children is a potentially devastating condition which is occasionally complicated by a clinical relapse. An autoimmune component has long been suspected in these relapses and recent findings suggest that antibodies against N-methyl-D-aspartate receptors (NMDARs) may be part of this mechanism. We here report an 11 months old girl with acute HSE and with negative NMDAR antibody serology at presentation who after an initial response to antiviral treatment deteriorated with seizures, abnormal movements, focal neurologic deficits and psychiatric symptoms. We show that this relapse occurred as production of NMDAR antibodies developed and that clinical improvement followed immunotherapy with a concomitant decrease in NMDAR antibody titers in CSF. She also developed a characteristic 15-20 Hz activity over both hemispheres which has been previously described as an electroencephalographic presentation of anti-NMDAR encephalitis. We conclude that relapse or persisting symptoms in HSE in children may represent an immune-mediated mechanism rather than a viral reactivation and that NMDAR antibodies should be analyzed as this may be of importance for the choice of therapy.
authors.affiliation: Neuropediatric Unit, Department of Women's and Children's Health, Karolinska Institutet, Sweden. Electronic address: ronny.wickstrom@ki.se.
authors.forename: Ronny
authors.initials: R
authors.lastname: Wickström
authors.affiliation: Neuropediatric Unit, Department of Women's and Children's Health, Karolinska Institutet, Sweden.
authors.forename: Asa
authors.initials: A
authors.lastname: Fowler
authors.affiliation: Department of Clinical Neurophysiology, Karolinska University Hospital, Sweden.
authors.forename: Gerald
authors.initials: G
authors.lastname: Cooray
authors.affiliation: Department of Immunology, Genetics and Pathology, Uppsala University, Sweden.
authors.forename: Alex
authors.initials: A
authors.lastname: Karlsson-Parra
authors.affiliation: Neuropediatric Unit, Department of Women's and Children's Health, Karolinska Institutet, Sweden.
authors.forename: Pernilla
authors.initials: P
authors.lastname: Grillner
mesh_terms: D060426:Anti-N-Methyl-D-Aspartate Receptor Encephalitis / Q000209:etiology* / Q000473:pathology / Q000821:virology*; D004569:Electroencephalography; D020803:Encephalitis, Herpes Simplex / Q000150:complications*; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging
keywords: Autoimmune; CNS; EEG; Extreme delta brush; Herpes simplex 1; Infant; Pediatric