Stiff person syndrome spectrum disorders; more than meets the eye.

Autoimmunity GAD65 Glutamic acid decarboxylase Stiff person spectrum disorders Stiff person syndrome

Journal

Journal of neuroimmunology
ISSN: 1872-8421
Titre abrégé: J Neuroimmunol
Pays: Netherlands
ID NLM: 8109498

Informations de publication

Date de publication:
15 08 2022
Historique:
received: 15 01 2022
revised: 21 05 2022
accepted: 07 06 2022
pubmed: 20 6 2022
medline: 8 7 2022
entrez: 19 6 2022
Statut: ppublish

Résumé

Stiff person syndrome spectrum disorders (SPSD) are a group of rare neuroimmunological disorders that often include painful spasms and rigidity. However, patients have highly heterogeneous signs and symptoms which may reflect different mechanistic disease processes. Understanding subsets of patients based on clinical phenotype may be important for prognosis and guiding treatment. The goal of this review is to provide updates on SPSD and its expanding clinical spectrum, prognostic markers, and treatment considerations. Further, we describe the current understanding in immunopathogenesis and highlight gaps in our knowledge appropriate for future research directions. Examples of revised diagnostic criteria for SPSD based on phenotype are also presented.

Identifiants

pubmed: 35717735
pii: S0165-5728(22)00110-2
doi: 10.1016/j.jneuroim.2022.577915
pmc: PMC9274902
mid: NIHMS1817271
pii:
doi:

Substances chimiques

Autoantibodies 0
Glutamate Decarboxylase EC 4.1.1.15

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

577915

Subventions

Organisme : Intramural NIH HHS
ID : Z99 NS999999
Pays : United States

Informations de copyright

Copyright © 2022 The Authors. Published by Elsevier B.V. All rights reserved.

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Auteurs

Scott D Newsome (SD)

Johns Hopkins University School of Medicine, Baltimore, MD, USA. Electronic address: snewsom2@jhmi.edu.

Tory Johnson (T)

Johns Hopkins University School of Medicine, Baltimore, MD, USA; Section of Infections of the Nervous System, NINDS, NIH, Bethesda, MD, USA.

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Classifications MeSH