Autoimmune encephalitis in children and adolescents.
Journal
Neurological research and practice
ISSN: 2524-3489
Titre abrégé: Neurol Res Pract
Pays: England
ID NLM: 101767802
Informations de publication
Date de publication:
2020
2020
Historique:
received:
08
10
2019
accepted:
28
11
2019
entrez:
16
12
2020
pubmed:
17
12
2020
medline:
17
12
2020
Statut:
epublish
Résumé
Autoimmune encephalitides with neural and glial antibodies have become an attractive field in neurology because the antibodies are syndrome-specific, explain the pathogenesis, indicate the likelihood of an underlying tumor, and often predict a good response to immunotherapy. The relevance and the management of antibody-associated encephalitides in the pediatric age group are to be discussed. Subacutely evolving, complex neuropsychiatric conditions that are otherwise unexplained should raise the suspicion of autoimmune encephalitis. Determination of autoantibodies is the key diagnostic step. It is recommended to study cerebrospinal fluid and serum in parallel to yield highest diagnostic sensitivity and specificity. The most frequently found antibodies are those against the N-methyl-D-asparate receptor, an antigen on the neural cell surface. The second most frequent antibody is directed against glutamic acid decarboxylase 65 kDa, an intracellular protein, often found in chronic conditions with questionable inflammatory activity. Immunotherapy is the mainstay of treatment in autoimmune encephalitides. Steroids, apheresis and intravenous immunoglobulin are first-line interventions. Rituximab or cyclophosphamide are given as second-line treatments. Patients with surface antibodies usually respond well to immunotherapy whereas cases with antibodies against intracellular antigens most often do not. With few exceptions, the experience in adult patients with autoimmune encephalitides can be applied to patients in the pediatric age range.
Sections du résumé
BACKGROUND
BACKGROUND
Autoimmune encephalitides with neural and glial antibodies have become an attractive field in neurology because the antibodies are syndrome-specific, explain the pathogenesis, indicate the likelihood of an underlying tumor, and often predict a good response to immunotherapy. The relevance and the management of antibody-associated encephalitides in the pediatric age group are to be discussed.
MAIN BODY
METHODS
Subacutely evolving, complex neuropsychiatric conditions that are otherwise unexplained should raise the suspicion of autoimmune encephalitis. Determination of autoantibodies is the key diagnostic step. It is recommended to study cerebrospinal fluid and serum in parallel to yield highest diagnostic sensitivity and specificity. The most frequently found antibodies are those against the N-methyl-D-asparate receptor, an antigen on the neural cell surface. The second most frequent antibody is directed against glutamic acid decarboxylase 65 kDa, an intracellular protein, often found in chronic conditions with questionable inflammatory activity. Immunotherapy is the mainstay of treatment in autoimmune encephalitides. Steroids, apheresis and intravenous immunoglobulin are first-line interventions. Rituximab or cyclophosphamide are given as second-line treatments. Patients with surface antibodies usually respond well to immunotherapy whereas cases with antibodies against intracellular antigens most often do not.
CONCLUSION
CONCLUSIONS
With few exceptions, the experience in adult patients with autoimmune encephalitides can be applied to patients in the pediatric age range.
Identifiants
pubmed: 33324910
doi: 10.1186/s42466-019-0047-8
pii: 47
pmc: PMC7650092
doi:
Types de publication
Journal Article
Review
Langues
eng
Pagination
4Informations de copyright
© The Author(s) 2020.
Déclaration de conflit d'intérêts
Competing interestsCGB obtained honoraria for speaking engagements from UCB (Monheim, Germany), Desitin (Hamburg, Germany), and Euroimmun (Lübeck, Germany). He receives research support from Deutsche Forschungsgemeinschaft (German Research Council, Bonn, Germany) and Gerd-Altenhof-Stiftung (Deutsches Stiftungs-Zentrum, Essen, Germany). CIB has no competing interests.
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