Accomplishment of Instrumental Activities of Daily Living and Its Relationship with Cognitive Functions in Adults with Myotonic Dystrophy Type 1 Childhood Phenotype: an Exploratory Study

Overview

Journal BMC Psychol

Publisher Biomed Central

Specialty Psychology

Date 2021 Apr 18

PMID 33865455

Citations 3

Authors

Marjolaine Tremblay

Samar Muslemani

Isabelle Cote

Cynthia Gagnon

Julie Fortin

Benjamin Gallais

Affiliations

Soon will be listed here.

Abstract

Background: The childhood phenotype of myotonic dystrophy type 1 (DM1) involves impaired cognitive functioning starting in infancy, which may compromise later on their ability to carry out instrumental activities of daily living (IADLs) necessary for living independently. The current study aims to document the ability to perform IADLs among adults with the childhood phenotype of DM1 and to explore its links to cognitive functioning.

Methods: This cross-sectional exploratory study was conducted among 11 individuals living with DM1. IADLs related to money management, home management & transportation and health & safety activities were assessed by the Independent Living Scale (ILS). Neuropsychological tests assessed participants' intellectual abilities and executive functioning. Associations were investigated using Spearman's rho correlation.

Results: Important difficulties were found in all three categories of IADLs, mostly in money management in which only 2/11 participants were scored as independent. 8/11 participants showed low to very low intellectual functioning and limit to impaired executive functioning. Apathy was also a common feature as 5/11 participants showed clinical level of apathy. A lower IQ was associated with greater difficulty in the home management & transportation subtest of the ILS.

Conclusions: Adults with the childhood phenotype of DM1 demonstrate relative dependence in regard to the following IADLs: money management and home management & transportation. Level of dependence is, at least partially, associated with cognitive impairments. The work relates to results from an exploratory study; thus, studies must be pursued to describe in more details difficulties experienced by this population.

Citing Articles

Cognitive impairment, neuroimaging abnormalities, and their correlations in myotonic dystrophy: a comprehensive review.

Wu Y, Wei Q, Lin J, Shang H, Ou R Front Cell Neurosci. 2024; 18:1369332.

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Longitudinal Changes in Neuropsychological Functioning in Japanese Patients with Myotonic Dystrophy Type 1: A Five Year Follow-Up Study.

Fujino H, Suwazono S, Ueda Y, Kobayashi M, Nakayama T, Imura O J Neuromuscul Dis. 2023; 10(6):1083-1092.

PMID: 37599536 PMC: 10657671. DOI: 10.3233/JND-230083.

Brain Pathogenesis and Potential Therapeutic Strategies in Myotonic Dystrophy Type 1.

Liu J, Guo Z, Yan X, Yang Y, Huang S Front Aging Neurosci. 2021; 13:755392.

PMID: 34867280 PMC: 8634727. DOI: 10.3389/fnagi.2021.755392.

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