The NIH Toolbox for cognitive surveillance in Duchenne muscular dystrophy.


Journal

Annals of clinical and translational neurology
ISSN: 2328-9503
Titre abrégé: Ann Clin Transl Neurol
Pays: United States
ID NLM: 101623278

Informations de publication

Date de publication:
09 2019
Historique:
received: 05 06 2019
revised: 15 07 2019
accepted: 16 07 2019
pubmed: 1 9 2019
medline: 19 5 2020
entrez: 1 9 2019
Statut: ppublish

Résumé

We performed a prospective, cross-sectional cognitive assessment in subjects with Duchenne Muscular Dystrophy (DMD) and their biological mothers. Thirty subjects with out-of-frame mutations in the dystrophin (DMD) gene, and 25 biological mothers were evaluated using the National Institutes of Health Toolbox Cognition Battery (NIHTB-CB). A parent completed the Behavior Rating Inventory of Executive Functioning (BRIEF), a standardized rating scale of executive functioning, for their child. Mothers completed self-reports of BRIEF and Neuro Quality-of-Life (NeuroQoL) Cognitive Function. Overall, the subjects with DMD scored approximately one standard deviation (SD) below age-corrected norms on the NIHTB-CB Total Cognition score. They scored 1.5 SD below age-corrected norms in Fluid Cognition, which evaluates the cognitive domains of executive function, working memory, episodic memory, attention, and processing speed. Their performance was consistent with age expectations (i.e., within 1 SD below age-corrected norms) in Crystalized Cognition, which evaluates vocabulary and reading. Subjects with DMD had higher T-scores in several domains of BRIEF, demonstrating greater difficulty in executive functioning. The biological mothers had overall average or above average T-scores on NIHTB-CB. Mothers who were carriers of DMD mutation performed lower overall compared to mothers who were not carriers of DMD mutation (Cohen's d = -1.1). Carrier mothers performed lower than average (1.5 SD) in Executive Function, measured by Flanker Inhibitory Control and Attention. Biological mothers scored within expected score ranges for adults in BRIEF and NeuroQoL. The NIHTB-CB, combined with standardized self-reported measures, can be a sensitive screening tool for cognitive surveillance in DMD.

Identifiants

pubmed: 31472009
doi: 10.1002/acn3.50867
pmc: PMC6764624
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

1696-1706

Subventions

Organisme : American Academy of Neuromuscular and Electrodiagnostic Medicine
ID : Award Number AWD00001989
Pays : International
Organisme : American Academy
ID : 2017
Pays : International
Organisme : American Academy
ID : 2019
Pays : International
Organisme : American Academy
ID : AWD00001989
Pays : International

Commentaires et corrections

Type : ErratumIn

Informations de copyright

© 2019 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals, Inc on behalf of American Neurological Association.

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Auteurs

Mathula Thangarajh (M)

Department of Neurology, Children's National Health System, District of Columbia, Washington.

Aaron J Kaat (AJ)

Department of Medical Social Sciences, Northwestern University, Chicago, Illinois.

Genila Bibat (G)

Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Johns Hopkins School of Medicine, Baltimore, Maryland.

Jennifer Mansour (J)

Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Johns Hopkins School of Medicine, Baltimore, Maryland.

Katherine Summerton (K)

Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Johns Hopkins School of Medicine, Baltimore, Maryland.

Anthony Gioia (A)

Departments of Psychiatry & Behavioral Science and Pediatrics, George Washington University School of Medicine, District of Columbia, Washington.

Carly Berger (C)

Departments of Psychiatry & Behavioral Science and Pediatrics, George Washington University School of Medicine, District of Columbia, Washington.

Kristina K Hardy (KK)

Departments of Psychiatry & Behavioral Science and Pediatrics, George Washington University School of Medicine, District of Columbia, Washington.

Kathryn R Wagner (KR)

Center for Genetic Muscle Disorders, Kennedy Krieger Institute, Johns Hopkins School of Medicine, Baltimore, Maryland.

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