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Lancet Neurol. 2016 Apr;15(4):391-404. doi: 10.1016/S1474-4422(15)00401-9. Epub 2016 Feb 20.

A clinical approach to diagnosis of autoimmune encephalitis.

Author information

1
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.
2
Department of Neurology, Erasmus Medical Center, Rotterdam, Netherlands.
3
Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA.
4
Department of Pediatrics, Alberta Children Hospital, Calgary, AB, Canada.
5
Epilepsy Centre Bethel, Krankenhaus Mara, Bielefeld, Germany.
6
Department of Pediatrics, McMaster Children's Hospital, McMaster University, Hamilton, ON, Canada.
7
Neuroimmunology Clinic, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA.
8
Neuroimmunology Group, Children's Hospital at Westmead, University of Sydney, Sydney, NSW, Australia.
9
Department of Neurology, University of California, San Francisco, CA, USA.
10
Division of Pediatric Infectious Diseases, Kaiser Permanente, Oakland Medical Center and University of California, San Francisco, CA, USA.
11
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.
12
Institute of Neurology, Medical University of Vienna, Vienna, Austria.
13
Department of Neurology, Kitasato University School of Medicine, Kanagawa, Japan.
14
Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford, UK.
15
Neuroimmunology, Institute of Clinical Chemistry, and Department of Neurology, University Medical Center Schleswig-Holstein, Kiel, Germany.
16
Department of Neurology, Charité Universitätsmedizin Berlin, Berlin, Germany; German Center for Neurodegenerative Disorders Berlin, Berlin, Germany.
17
Department of Neurology, Cleveland Clinic Foundation, Cleveland, OH, USA.
18
Clinical Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria.
19
Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain.
20
Department of Pediatric Neurology, Children's Hospital Datteln, Witten/Herdecke University, Datteln, Germany.
21
Neuroimmunology Program, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain; Service of Neurology, Hospital Clinic, Barcelona, Spain.
22
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
23
Institute of Clinical Chemistry and Department of Neurology, University Hospital Schleswig-Holstein, Lübeck, Germany.
24
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.

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.

PMID:
26906964
PMCID:
PMC5066574
DOI:
10.1016/S1474-4422(15)00401-9
[Indexed for MEDLINE]
Free PMC Article

Conflict of interest statement

Declaration of interests FG receives royalties from licensing fees to Euroimmun for the use of IgLON5 as a diagnostic test. MJT has received research funding for consultancy work for MedImmune, and a travel grant for Sun Pharma. CGB has given scientific advice to Eisai and UCB; undertaken industry-funded travel with support from Eisai, UCB, Desitin, and Grifols; obtained honoraria for speaking engagements from Eisai, UCB, Desitin, Diamed, Fresenius Medical Care; and received research support from Astellas Pharma, Octapharma, Diamed, and Fresenius Medical Care. CGB is an employee of Krankenhaus Mara, Bielefeld, Germany, which runs a laboratory for the detection of autoantibodies including those described in this paper; external senders are charged for antibody diagnostics. RCD has received research funding from the Star Scientific Foundation and Pfizer Neuroscience and speaker’s honoraria from Biogen Idec and Bristol-Myers Squibb. JMG has received compensation for medical legal consulting and for consulting on a scientific advisory board for Medimmune and Roche; he has received research funding through the University of California, San Francisco, USA, from Quest Diagnostic for work on a dementia care pathway. MG receives grants from Quest Diagnostics and has received personal fees for consultancy work from MedaCorp, Gerson-Lehman Group, Best Doctors, Advance Medical, Inc, and Optio LLC. JH receives royalties from licensing fees to Athena Diagnostics, Euroimmun, and ravo Diagnostika for a patent for the use of CV2/CRMP5 as diagnostic tests. SRI receives royalties from licensing fees to Euroimmun for patents for the use of LGI1, CASPR2, and contactin-2 as autoantibody tests. EL has received speaker’s honoraria and consultancy fees from Grifols, and consultancy fees from Medimmune. FL has received speaker’s honoraria from Grifols, Teva, and Biogen Idec and is employed by University Medical Center Schleswig-Holstein, Kiel, Germany, which offers commercial antibody testing without any personal reimbursements. MR reports that his employers, the University Hospital and Medical University of Innsbruck, Austria, receive payments for antibody assays (NMDA receptor, AQP4, and other autoantibodies) and for AQP4 antibody validation experiments organised by Euroimmun. MRR receives royalties from licensing fees to Euroimmun for a patent for the use of NMDA receptor as an autoantibody test, and from licensing fees to Athena Diagnostics for a patent for the use of Ma2. AS has received compensation for consulting services and speaker honoraria from Bayer-Schering, Merck-Serono, Biogen Idec, Sanofi-Aventis, Teva, and Novartis. AVe reports personal fees from Medimmune. AVi receives royalties from licensing fees to Euroimmun for the use of LGI1 and CASPR2 as diagnostic tests. PW receives royalties for the use of LGI1 and CASPR2 as autoantibody diagnostic tests; is a named inventor on a patent for the use of GABAA receptor as an autoantibody test; and has received speaker honoraria from Biogen Idec and Euroimmun. JD receives royalties from licensing fees to Athena Diagnostics for a patent for the use of Ma2 as an autoantibody test; licensing fees to Euroimmun for patents for the use of NMDA receptor and GABAB receptor as autoantibody tests; licensing fees for the use of DPPX, GABAA receptor, and IgLON5 antibodies as diagnostic tests; and has received a research grant from Euroimmun. RB, SB, TC, IC, CAG, RH, TI, HP, AR-G, KR, and K-PW declare no competing interests. None of the funding sources had any influence in the preparation of this Position Paper.

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