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Neuropediatrics 2023; 54(01): 014-019
DOI: 10.1055/s-0042-1757706
Original Article

Autoimmune Encephalitis with Autoantibodies to NMDAR1 following Herpes Encephalitis in Children and Adolescents

Authors

  • Annegret Quade 

    1   Department of Pediatrics, Division of Neuropediatrics and Social Pediatrics, Medical Faculty, RWTH Aachen University, Aachen, Germany

  • Kevin Rostasy

    2   Department of Paediatric Neurology, Children's Hospital Datteln, Witten/Herdecke University, Witten, Germany

  • Ronny Wickström

    3   Department of Women's and Children's Health, Division of Neuropediatrics, Karolinska Institute, Solna, Sweden

  • Ömer Faruk Aydin

    4   Department of Pediatric Neurology, Medical Faculty, Ondokuz Mayis University, Turkey

  • Stefano Sartori

    5   Department of Women's and Children's Health, Paediatric Neurology and Neurophysiology Unit, University Hospital of Padua, Padua, Italy and Neuroimmunology Group, Paediatric Research Institute “Città della Speranza,” Padova, Italy

  • Margherita Nosadini

    5   Department of Women's and Children's Health, Paediatric Neurology and Neurophysiology Unit, University Hospital of Padua, Padua, Italy and Neuroimmunology Group, Paediatric Research Institute “Città della Speranza,” Padova, Italy

  • Ellen Knierim

    6   Department of Neuropediatrics, Charité–Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin, Germany

  • Gerhard Kluger

    7   Clinic for Neuropediatrics and Neurorehabilitation, Epilepsy Center for Children and Adolescents, Schön Klinik, Vogtareuth, Germany
    8   Research Institute “Rehabilitation-Transition-Palliation,” PMU Salzburg, Austria

  • Rudolf Korinthenberg 

    9   Department of Neuropediatrics and Muscular Disorders, Medical Faculty, University of Freiburg, Freiburg, Germany

  • Burkhard Stüve

    10   Department of Neuropediatrics, Children's Hospital Siegen, Germany

  • Stephan Waltz

    11   Neuropediatric Department, Children's Hospital Cologne, Germany

  • Steffen Leiz

    12   Department of Pediatrics, Neuropediatrics, Klinikum Dritter Orden München – Nymphenburg, Germany

  • Martin Häusler 

    1   Department of Pediatrics, Division of Neuropediatrics and Social Pediatrics, Medical Faculty, RWTH Aachen University, Aachen, Germany
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Abstract

Herpes simplex virus (HSV) type 1 is a frequent pathogen causing infectious encephalitis (HSVE). Early treatment with intravenous acyclovir has led to a significant decrease in mortality. However, especially in children, deterioration during or after HSVE may occur without any evidence of HSV reactivation or improvement following repeated antiviral therapy. Here, we report 15 patients (age range 3 months to 15 years) who suffered from autoimmune encephalitis with autoantibodies to NMDAR1 following Herpes encephalitis, presenting with movement abnormalities (young children) or neuropsychiatric symptoms (older children) as major complaints, respectively. The diagnosis was based on positive cerebrospinal fluid (CSF) and/or serum anti-NMDAR-antibodies with two children showing only positive CSF antibody findings. The time lag between first symptoms and diagnosis of autoimmune encephalitis was significantly longer than between first symptoms and diagnosis of HSVE (p <0.01). All patients improved during immunosuppressive treatment, during which plasmapheresis or rituximab treatments were applied in 11 patients, irrespective of their age. Despite immunotherapy, no patients relapsed with HSVE. Early diagnosis and treatment of autoimmune encephalitis after HSVE may be associated with a better outcome so that high clinical awareness and routine testing for anti-NMDAR-antibodies after HSVE seems advisable. If autoimmune encephalitis is suspected, antibody testing should also be performed on CSF if negative in serum.

Keywords

Herpes simplex encephalitis - autoimmune encephalitis - anti- NMDAR antibodies

Supplementary Material



Publication History

Received: 11 November 2021

Accepted: 12 August 2022

Article published online:
21 December 2022

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