Adult onset familiar dystonia-plus syndrome: A novel presentation of IRF2BPL-associated neurodegeneration.


Journal

Parkinsonism & related disorders
ISSN: 1873-5126
Titre abrégé: Parkinsonism Relat Disord
Pays: England
ID NLM: 9513583

Informations de publication

Date de publication:
01 2022
Historique:
received: 23 06 2021
revised: 23 10 2021
accepted: 31 10 2021
pubmed: 6 12 2021
medline: 19 4 2022
entrez: 5 12 2021
Statut: ppublish

Résumé

Pathogenic variants of the IRF2BPL gene have been mostly associated with early onset epileptic encephalopathy. Movement disorders such as dystonia and ataxia were also reported, with symptoms mainly developing between childhood and adolescence. Here we describe a family with several members affected by a late onset dystonic and ataxic progressive syndrome, caused by a novel heterozygous pathogenic variant in the IRF2BPL gene.

Identifiants

pubmed: 34864472
pii: S1353-8020(21)00396-5
doi: 10.1016/j.parkreldis.2021.10.033
pii:
doi:

Substances chimiques

Carrier Proteins 0
IRF2BPL protein, human 0
Nuclear Proteins 0

Types de publication

Case Reports Letter Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

22-24

Informations de copyright

Copyright © 2021 Elsevier Ltd. All rights reserved.

Auteurs

Francesca Antonelli (F)

Neurological Clinic, Neurosciences Department, University of Modena and Reggio Emilia, Reggio Emilia, Italy. Electronic address: antonelli.f@gmail.com.

Gaetano Grieco (G)

Neurogenetics Research Center, IRCCS Mondino Foundation, Pavia, Italy.

Francesco Cavallieri (F)

Neurology Unit, Neuromotor & Rehabilitation Department, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy; Clinical and Experimental Medicine PhD Program, University of Modena and Reggio Emilia, Italy.

Antonella Casella (A)

Neurogenetics Research Center, IRCCS Mondino Foundation, Pavia, Italy; Department of Molecular Medicine, University of Pavia, Pavia, Italy.

Enza Maria Valente (EM)

Neurogenetics Research Center, IRCCS Mondino Foundation, Pavia, Italy; Department of Molecular Medicine, University of Pavia, Pavia, Italy.

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Classifications MeSH