Clinical and Prognostic Value of Immunogenetic Characteristics in Anti-LGI1 Encephalitis.
Journal
Neurology(R) neuroimmunology & neuroinflammation
ISSN: 2332-7812
Titre abrégé: Neurol Neuroimmunol Neuroinflamm
Pays: United States
ID NLM: 101636388
Informations de publication
Date de publication:
05 2021
05 2021
Historique:
received:
08
09
2020
accepted:
04
01
2021
entrez:
13
4
2021
pubmed:
14
4
2021
medline:
16
12
2021
Statut:
epublish
Résumé
Antibodies against leucine-rich glioma-inactivated 1 (LGI1-Abs) characterize a limbic encephalitis (LE) strongly associated with HLA-DRB1*07:01, although some patients lack LGI1-Abs in CSF or do not carry this allele. Whether they represent a different subtype of disease or have different prognoses is unclear. Retrospective analysis of clinical features, IgG isotypes, and outcome according to LGI1-Ab CSF positivity and DRB1*07:01 in a cohort of anti-LGI1 LE patients. Patients with LGI1-Abs detected in both CSF and serum (105/134, 78%) were compared with those who were CSF negative (29/134, 22%). Both groups had similar clinical features and serum levels, but CSF-positive patients had shorter diagnostic delay, more frequently hyponatremia, inflammatory CSF, and abnormal MRI ( LE without CSF LGI1-Abs is clinically indistinguishable and likely reflects just a lesser LGI1-Ab production. HLA association is sex and age biased and presents clinical particularities, suggesting subtle differences in the immune response. Long-term outcome depends mostly on demographic characteristics and the intensity of the intrathecal synthesis.
Identifiants
pubmed: 33848259
pii: 8/3/e974
doi: 10.1212/NXI.0000000000000974
pmc: PMC7938443
pii:
doi:
Substances chimiques
Autoantibodies
0
HLA Antigens
0
Immunoglobulin G
0
Intracellular Signaling Peptides and Proteins
0
LGI1 protein, human
0
anti-leucine-rich glioma-inactivated 1 autoantibody
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Informations de copyright
Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
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