Clinical approach to the diagnosis of autoimmune encephalitis in the pediatric patient

Tania Cellucci; Heather Van Mater; Francesc Graus; Eyal Muscal; William Gallentine; Marisa S Klein-Gitelman; Susanne M Benseler; Jennifer Frankovich; Mark P Gorman; Keith Van Haren; Josep Dalmau; Russell C Dale

doi:10.1212/NXI.0000000000000663

Clinical approach to the diagnosis of autoimmune encephalitis in the pediatric patient

Neurol Neuroimmunol Neuroinflamm. 2020 Jan 17;7(2):e663. doi: 10.1212/NXI.0000000000000663. Print 2020 Mar.

Affiliations

¹ From the Department of Pediatrics (T.C.), McMaster Children's Hospital, McMaster University, Hamilton, Ontario, Canada; Department of Pediatrics (H.V.M.), Duke University, Durham, NC; Neuroimmunology Program (F.G., J.D.), Institut d'Investigacions Biomèdiques August Pi I Sunyer, Barcelona, Spain; Department of Pediatrics (E.M.), Baylor College of Medicine, Houston, TX; Department of Neurology (W.G., K.V.H.), Lucile Packard Children's Hospital, Stanford University, Palo Alto, CA; Division of Rheumatology (M.S.K.-G.), Ann & Robert H, Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, IL; Department of Pediatrics (S.M.B.), Alberta Children's Hospital, University of Calgary, Canada; Division of Pediatric Allergy, Immunology and Rheumatology (J.F.), Lucile Packard Children's Hospital, Stanford University, Palo Alto, CA; Department of Neurology (M.P.G.), Boston Children's Hospital, Harvard University, MA; Department of Neurology (J.D.), University of Pennsylvania, Philadelphia; Catalan Institution for Research and Advanced Studies (ICREA) (J.D.); Neuroimmunology Group (R.C.D.), Children's Hospital at Westmead, Faculty of Medicine and Health, University of Sydney, New South Wales, Australia.
² From the Department of Pediatrics (T.C.), McMaster Children's Hospital, McMaster University, Hamilton, Ontario, Canada; Department of Pediatrics (H.V.M.), Duke University, Durham, NC; Neuroimmunology Program (F.G., J.D.), Institut d'Investigacions Biomèdiques August Pi I Sunyer, Barcelona, Spain; Department of Pediatrics (E.M.), Baylor College of Medicine, Houston, TX; Department of Neurology (W.G., K.V.H.), Lucile Packard Children's Hospital, Stanford University, Palo Alto, CA; Division of Rheumatology (M.S.K.-G.), Ann & Robert H, Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, IL; Department of Pediatrics (S.M.B.), Alberta Children's Hospital, University of Calgary, Canada; Division of Pediatric Allergy, Immunology and Rheumatology (J.F.), Lucile Packard Children's Hospital, Stanford University, Palo Alto, CA; Department of Neurology (M.P.G.), Boston Children's Hospital, Harvard University, MA; Department of Neurology (J.D.), University of Pennsylvania, Philadelphia; Catalan Institution for Research and Advanced Studies (ICREA) (J.D.); Neuroimmunology Group (R.C.D.), Children's Hospital at Westmead, Faculty of Medicine and Health, University of Sydney, New South Wales, Australia. Russell.dale@health.nsw.gov.au.

Abstract

Objective: Autoimmune encephalitis (AE) is an important and treatable cause of acute encephalitis. Diagnosis of AE in a developing child is challenging because of overlap in clinical presentations with other diseases and complexity of normal behavior changes. Existing diagnostic criteria for adult AE require modification to be applied to children, who differ from adults in their clinical presentations, paraclinical findings, autoantibody profiles, treatment response, and long-term outcomes.

Methods: A subcommittee of the Autoimmune Encephalitis International Working Group collaborated through conference calls and email correspondence to consider the pediatric-specific approach to AE. The subcommittee reviewed the literature of relevant AE studies and sought additional input from other expert clinicians and researchers.

Results: Existing consensus criteria for adult AE were refined for use in children. Provisional pediatric AE classification criteria and an algorithm to facilitate early diagnosis are proposed. There is also discussion about how to distinguish pediatric AE from conditions within the differential diagnosis.

Conclusions: Diagnosing AE is based on the combination of a clinical history consistent with pediatric AE and supportive diagnostic testing, which includes but is not dependent on antibody testing. The proposed criteria and algorithm require validation in prospective pediatric cohorts.

MeSH terms

Adolescent
Algorithms*
Autoantibodies / blood
Autoantibodies / cerebrospinal fluid
Autoantibodies / metabolism*
Autoimmune Diseases of the Nervous System / diagnosis*
Autoimmune Diseases of the Nervous System / immunology
Autoimmune Diseases of the Nervous System / metabolism
Autoimmune Diseases of the Nervous System / physiopathology
Child
Consensus
Diagnosis, Differential
Encephalitis / diagnosis*
Encephalitis / immunology
Encephalitis / metabolism
Encephalitis / physiopathology
Humans
Magnetic Resonance Imaging
Practice Guidelines as Topic / standards*

Substances

Autoantibodies