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Management of antibody-mediated autoimmune encephalitis in adults and children: literature review and consensus-based practical recommendations

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Abstract

Autoimmune encephalitis associated with antibodies against neuronal surface targets (NSAE) are rare but still underrecognized conditions that affect adult and pediatric patients. Clinical guidelines have recently been published with the aim of providing diagnostic clues regardless of antibody status. These syndromes are potentially treatable but the choice of treatment and its timing, as well as differential diagnoses, long-term management, and clinical and paraclinical follow-up, remain major challenges. In the absence of evidence-based guidelines, management of these conditions is commonly based on single-center expertise.

Taking into account different published expert recommendations in addition to the multicenter experience of the Italian Working Group on Autoimmune Encephalitis, both widely accepted and critical aspects of diagnosis, management and particularly of immunotherapy for NSAE have been reviewed and are discussed.

Finally, we provide consensus-based practical advice for managing hospitalization and follow-up of patients with NSAE.

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The authors thank Joanne Fleming for reviewing the manuscript.

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Authors and Affiliations

  1. Department of Neurology, Ospedale San Bortolo, Azienda ULSS8 Berica, Via Rodolfi 37, 36100, Vicenza, Italy

    Luigi Zuliani & Francesco Perini

  2. Neuroimmunology Group, Pediatric Research Institute “Città della Speranza”, Padova, Italy

    Luigi Zuliani, Margherita Nosadini, Marco Zoccarato, Piera De Gaspari & Stefano Sartori

  3. Department of Women’s and Children’s Health, Paediatric Neurology and Neurophysiology Unit, University Hospital of Padua, Padua, Italy

    Margherita Nosadini & Stefano Sartori

  4. Neuroimmunology Laboratory, IRCCS Mondino Foundation, Pavia, Italy

    Matteo Gastaldi & Diego Franciotta

  5. Institut d’Investigacions Biomediques August Pi i Sunyer, Hospital Clınic, University of Barcelona, Barcelona, Spain

    Marianna Spatola

  6. Department of Neuroscience, Fondazione Policlinico Universitario ‘A.Gemelli’ IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy

    Raffaele Iorio & Amelia Evoli

  7. Deparment of Neurology, Azienda ULSS Euganea, Padua, Italy

    Marco Zoccarato

  8. Department of Neurological, Biomedical and Movement Sciences, University of Verona, Verona, Italy

    Sara Mariotto & Sergio Ferrari

  9. Department of Neurology, Ospedale Santa Chiara, Trento, Italy

    Bruno Giometto

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Zuliani, L., Nosadini, M., Gastaldi, M. et al. Management of antibody-mediated autoimmune encephalitis in adults and children: literature review and consensus-based practical recommendations. Neurol Sci 40, 2017–2030 (2019). https://doi.org/10.1007/s10072-019-03930-3

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