title: A clinical approach to diagnosis of autoimmune encephalitis.
abstract: Encephalitis is a severe inflammatory disorder of the brain with many possible causes and a complex differential diagnosis. Advances in autoimmune encephalitis research in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to these disorders. However, existing criteria for autoimmune encephalitis are too reliant on antibody testing and response to immunotherapy, which might delay the diagnosis. We reviewed the literature and gathered the experience of a team of experts with the aims of developing a practical, syndrome-based diagnostic approach to autoimmune encephalitis and providing guidelines to navigate through the differential diagnosis. Because autoantibody test results and response to therapy are not available at disease onset, we based the initial diagnostic approach on neurological assessment and conventional tests that are accessible to most clinicians. Through logical differential diagnosis, levels of evidence for autoimmune encephalitis (possible, probable, or definite) are achieved, which can lead to prompt immunotherapy.
authors.affiliation: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain; Service of Neurology, Hospital Clinic, Barcelona, Spain. Electronic address: fgraus@clinic.ub.es.
authors.forename: Francesc
authors.initials: F
authors.lastname: Graus
authors.affiliation: Department of Neurology, Erasmus Medical Center, Rotterdam, Netherlands.
authors.forename: Maarten J
authors.initials: MJ
authors.lastname: Titulaer
authors.affiliation: Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Ramani
authors.initials: R
authors.lastname: Balu
authors.affiliation: Department of Pediatrics, Alberta Children Hospital, Calgary, AB, Canada.
authors.forename: Susanne
authors.initials: S
authors.lastname: Benseler
authors.affiliation: Epilepsy Centre Bethel, Krankenhaus Mara, Bielefeld, Germany.
authors.forename: Christian G
authors.initials: CG
authors.lastname: Bien
authors.affiliation: Department of Pediatrics, McMaster Children's Hospital, McMaster University, Hamilton, ON, Canada.
authors.forename: Tania
authors.initials: T
authors.lastname: Cellucci
authors.affiliation: Neuroimmunology Clinic, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Irene
authors.initials: I
authors.lastname: Cortese
authors.affiliation: Neuroimmunology Group, Children's Hospital at Westmead, University of Sydney, Sydney, NSW, Australia.
authors.forename: Russell C
authors.initials: RC
authors.lastname: Dale
authors.affiliation: Department of Neurology, University of California, San Francisco, CA, USA.
authors.forename: Jeffrey M
authors.initials: JM
authors.lastname: Gelfand
authors.affiliation: Department of Neurology, University of California, San Francisco, CA, USA.
authors.forename: Michael
authors.initials: M
authors.lastname: Geschwind
authors.affiliation: Division of Pediatric Infectious Diseases, Kaiser Permanente, Oakland Medical Center and University of California, San Francisco, CA, USA.
authors.forename: Carol A
authors.initials: CA
authors.lastname: Glaser
authors.affiliation: French Reference Center on Paraneoplastic Neurological Syndrome, Hospices Civils De Lyon, Hôpital Neurologique, Inserm U1028, CNRS UMR 5292, Lyon's Neurosciences Research Center, Université Claude-Bernard Lyon-1, Lyon, France.
authors.forename: Jerome
authors.initials: J
authors.lastname: Honnorat
authors.affiliation: Institute of Neurology, Medical University of Vienna, Vienna, Austria.
authors.forename: Romana
authors.initials: R
authors.lastname: Höftberger
authors.affiliation: Department of Neurology, Kitasato University School of Medicine, Kanagawa, Japan.
authors.forename: Takahiro
authors.initials: T
authors.lastname: Iizuka
authors.affiliation: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.
authors.forename: Sarosh R
authors.initials: SR
authors.lastname: Irani
authors.affiliation: Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Eric
authors.initials: E
authors.lastname: Lancaster
authors.affiliation: Neuroimmunology, Institute of Clinical Chemistry, and Department of Neurology, University Medical Center Schleswig-Holstein, Kiel, Germany.
authors.forename: Frank
authors.initials: F
authors.lastname: Leypoldt
authors.affiliation: Department of Neurology, Charité Universitätsmedizin Berlin, Berlin, Germany; German Center for Neurodegenerative Disorders Berlin, Berlin, Germany.
authors.forename: Harald
authors.initials: H
authors.lastname: Prüss
authors.affiliation: Department of Neurology, Cleveland Clinic Foundation, Cleveland, OH, USA.
authors.forename: Alexander
authors.initials: A
authors.lastname: Rae-Grant
authors.affiliation: Clinical Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria.
authors.forename: Markus
authors.initials: M
authors.lastname: Reindl
authors.affiliation: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain.
authors.forename: Myrna R
authors.initials: MR
authors.lastname: Rosenfeld
authors.affiliation: Department of Pediatric Neurology, Children's Hospital Datteln, Witten/Herdecke University, Datteln, Germany.
authors.forename: Kevin
authors.initials: K
authors.lastname: Rostásy
authors.affiliation: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain; Service of Neurology, Hospital Clinic, Barcelona, Spain.
authors.forename: Albert
authors.initials: A
authors.lastname: Saiz
authors.affiliation: Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
authors.forename: Arun
authors.initials: A
authors.lastname: Venkatesan
authors.affiliation: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.
authors.forename: Angela
authors.initials: A
authors.lastname: Vincent
authors.affiliation: Institute of Clinical Chemistry and Department of Neurology, University Hospital Schleswig-Holstein, Lübeck, Germany.
authors.forename: Klaus-Peter
authors.initials: KP
authors.lastname: Wandinger
authors.affiliation: Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.
authors.forename: Patrick
authors.initials: P
authors.lastname: Waters
authors.affiliation: Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain; Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA; Institució Catalana de Recerca i Estudis Avançats (ICREA), Barcelona, Spain. Electronic address: jdalmau@clinic.ub.es.
authors.forename: Josep
authors.initials: J
authors.lastname: Dalmau
mesh_terms: D020274:Autoimmune Diseases of the Nervous System / Q000175:diagnosis*; D004660:Encephalitis / Q000175:diagnosis*; D006801:Humans