A clinical approach to diagnosis of autoimmune encephalitis

Francesc Graus; Maarten J. Titulaer; Ramani Balu; Susanne Benseler; Christian G. Bien; Tania Cellucci; Irene Cortese; Russell C. Dale; Jeffrey M. Gelfand; Michael Geschwind; Carol A. Glaser; Jerome Honnorat; Romana Höftberger; Takahiro Iizuka; Sarosh R. Irani; Eric Lancaster; Frank Leypoldt; Harald Prüss; Alexander Rae-Grant; Markus Reindl; Myrna R. Rosenfeld; Kevin Rostásy; Albert Saiz; Arun Venkatesan; Angela Vincent; Klaus Peter Wandinger; Patrick Waters; Joseph Dalmau

doi:10.1016/S1474-4422(15)00401-9

A clinical approach to diagnosis of autoimmune encephalitis

Francesc Graus^*, Maarten J. Titulaer, Ramani Balu, Susanne Benseler, Christian G. Bien, Tania Cellucci, Irene Cortese, Russell C. Dale, Jeffrey M. Gelfand, Michael Geschwind, Carol A. Glaser, Jerome Honnorat, Romana Höftberger, Takahiro Iizuka, Sarosh R. Irani, Eric Lancaster, Frank Leypoldt, Harald Prüss, Alexander Rae-Grant, Markus ReindlMyrna R. Rosenfeld, Kevin Rostásy, Albert Saiz, Arun Venkatesan, Angela Vincent, Klaus Peter Wandinger, Patrick Waters, Joseph Dalmau

^*Corresponding author for this work

Institute of Clinical Chemistry

3324 Citations (Scopus)

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.

Original language	English
Journal	The Lancet Neurology
Volume	15
Issue number	4
Pages (from-to)	391-404
Number of pages	14
ISSN	1474-4422
DOIs	https://doi.org/10.1016/S1474-4422(15)00401-9
Publication status	Published - 01.04.2016

Funding

We thank the Autoimmune Encephalitis Alliance (USA), the Encephalitis Society (UK), the Anti-NMDA Receptor Encephalitis Foundation Inc (Canada), and the Anti-NMDA Receptor Encephalitis Patient Initiative (Germany) for disseminating information, helping patients and families, and promoting research in autoimmune encephalitis. FG was supported in part by grant 20141830 Fundació la Marató TV3. MJT has been supported by an Erasmus fellowship, the Netherlands Organisation for Scientific Research (Veni-incentive), and a grant from the Dutch Epilepsy Foundations (NEF project 14–19). RCD has received research funding from the National Health and Medical Research Council, MS Research Australia, the Tourette Syndrome Association, the University of Sydney, and the Petre Foundation. MG receives grants from the National Institute on Aging; has received grants from CurePSP and the Tau Consortium; and has received speaker''s fees and research funding from Grand Round Lectures, and the Michael J Homer Family Fund. PW is supported by the National Health Service National Specialised Commissioning Group for Neuromyelitis Optica, UK, and the National Institute for Health Research Oxford Biomedical Research Centre, and has received travel grants from the Guthy-Jackson Charitable Foundation. JD was supported by the Instituto Carlos III (FIS 14/00203) grant, National Institutes of Health RO1NS077851 grant, and Fundació Cellex.

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Graus, F., Titulaer, M. J., Balu, R., Benseler, S., Bien, C. G., Cellucci, T., Cortese, I., Dale, R. C., Gelfand, J. M., Geschwind, M., Glaser, C. A., Honnorat, J., Höftberger, R., Iizuka, T., Irani, S. R., Lancaster, E., Leypoldt, F., Prüss, H., Rae-Grant, A., ... Dalmau, J. (2016). A clinical approach to diagnosis of autoimmune encephalitis. The Lancet Neurology, 15(4), 391-404. https://doi.org/10.1016/S1474-4422(15)00401-9

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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.",

author = "Francesc Graus and Titulaer, \{Maarten J.\} and Ramani Balu and Susanne Benseler and Bien, \{Christian G.\} and Tania Cellucci and Irene Cortese and Dale, \{Russell C.\} and Gelfand, \{Jeffrey M.\} and Michael Geschwind and Glaser, \{Carol A.\} and Jerome Honnorat and Romana H{\"o}ftberger and Takahiro Iizuka and Irani, \{Sarosh R.\} and Eric Lancaster and Frank Leypoldt and Harald Pr{\"u}ss and Alexander Rae-Grant and Markus Reindl and Rosenfeld, \{Myrna R.\} and Kevin Rost{\'a}sy and Albert Saiz and Arun Venkatesan and Angela Vincent and Wandinger, \{Klaus Peter\} and Patrick Waters and Joseph Dalmau",

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doi = "10.1016/S1474-4422(15)00401-9",

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Graus, F, Titulaer, MJ, Balu, R, Benseler, S, Bien, CG, Cellucci, T, Cortese, I, Dale, RC, Gelfand, JM, Geschwind, M, Glaser, CA, Honnorat, J, Höftberger, R, Iizuka, T, Irani, SR, Lancaster, E, Leypoldt, F, Prüss, H, Rae-Grant, A, Reindl, M, Rosenfeld, MR, Rostásy, K, Saiz, A, Venkatesan, A, Vincent, A, Wandinger, KP, Waters, P & Dalmau, J 2016, 'A clinical approach to diagnosis of autoimmune encephalitis', The Lancet Neurology, vol. 15, no. 4, pp. 391-404. https://doi.org/10.1016/S1474-4422(15)00401-9

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AU - Graus, Francesc

AU - Titulaer, Maarten J.

AU - Balu, Ramani

AU - Benseler, Susanne

AU - Bien, Christian G.

AU - Cellucci, Tania

AU - Cortese, Irene

AU - Dale, Russell C.

AU - Gelfand, Jeffrey M.

AU - Geschwind, Michael

AU - Glaser, Carol A.

AU - Honnorat, Jerome

AU - Höftberger, Romana

AU - Iizuka, Takahiro

AU - Irani, Sarosh R.

AU - Lancaster, Eric

AU - Leypoldt, Frank

AU - Prüss, Harald

AU - Rae-Grant, Alexander

AU - Reindl, Markus

AU - Rosenfeld, Myrna R.

AU - Rostásy, Kevin

AU - Saiz, Albert

AU - Venkatesan, Arun

AU - Vincent, Angela

AU - Wandinger, Klaus Peter

AU - Waters, Patrick

AU - Dalmau, Joseph

PY - 2016/4/1

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N2 - 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.

AB - 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.

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DO - 10.1016/S1474-4422(15)00401-9

M3 - Scientific review articles

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AN - SCOPUS:84960486545

SN - 1474-4422

VL - 15

SP - 391

EP - 404

JO - The Lancet Neurology

JF - The Lancet Neurology

IS - 4

ER -

A clinical approach to diagnosis of autoimmune encephalitis

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