Cognitive profile of patients with facioscapulohumeral muscular dystrophy.

Autor:	Dos Santos VB; Postgraduate Program in Rehabilitation Sciences, Universidade Federal de Ciências da Saúde de Porto Alegre - Porto Alegre, RS, Brazil., Saute JAM; Department of Internal Medicine, Universidade Federal do Rio Grande do Sul - Porto Alegre, RS, Brazil.; Postgraduate Program in Medicine: Medical Sciences, Universidade Federal do Rio Grande do Sul - Porto Alegre, RS, Brazil., Jacinto-Scudeiro LA; Postgraduate Program in Medicine: Medical Sciences, Universidade Federal do Rio Grande do Sul - Porto Alegre, RS, Brazil., Ayres A; Postgraduate Program in Health Sciences, Universidade Federal de Ciências da Saúde de Porto Alegre -Porto Alegre, RS, Brazil., Rech RS; Postgraduate Program in Epidemiology, Universidade Federal de Ciências da Saúde de Porto Alegre -Porto Alegre, RS, Brazil., de Oliveira AA; Postgraduate Program in Rehabilitation Sciences, Universidade Federal de Ciências da Saúde de Porto Alegre - Porto Alegre, RS, Brazil., Olchik MR; Postgraduate Program in Medicine: Medical Sciences, Universidade Federal do Rio Grande do Sul - Porto Alegre, RS, Brazil.; Department of Surgery and Orthopedics, Universidade Federal do Rio Grande do Sul - Porto Alegre, RS, Brazil.
Jazyk:	angličtina
Zdroj:	Dementia & neuropsychologia [Dement Neuropsychol] 2021 Oct-Dec; Vol. 15 (4), pp. 541-547.
DOI:	10.1590/1980-57642021dn15-040015
Abstrakt:	Although it is predominantly a muscular disease, impairments in the central nervous system in patients with facioscapulohumeral muscular dystrophy (FSHD) have been described in the literature. Objective: To describe the cognitive profile of patients with FSHD and to correlate the impairments found with clinical variables and quality of life. Methods: Cross-sectional and case-control study that evaluated FSHD patients using a series of cognitive assessments (Mini-Mental State Examination - MMSE, Montreal Cognitive Assessment - MoCA, verbal fluency with phonological restriction - FAS, categorical verbal fluency - FAS-cat, trail-making test - TMT, and Rey's Verbal Auditory Learning Test); a neurological severity scale (Gardner-Medwin-Walton - GMWS); and a quality of life measurement tool (Medical Outcomes Study 36-Item Short-Form Health Survey). Results: Individuals with FSHD (13) and healthy controls (26) were paired by gender and age. Significant differences between case and control groups were found in MMSE, TMT A, and A7 (p≤0.05) and MOCA (p≤0.001) performances. A positive correlation was verified in long-term memory impairments and the age in which symptoms appear (r=-0.593, p=0.033). Regarding quality of life assessment, the emotional domain correlated to MEEM (r=0.657, p=0.015), TMT A (r=-0.601, p=0.030), and A7 (r=0.617, p=0.025) performances. Conclusions: Individuals with FSHD presented mild impairments in the performance of tasks that involve attention, planning, and long-term memory functions. Those impairments were associated neither with the disease duration nor with its neurological severity. Competing Interests: Disclosure: The authors report no conflicts of interest.
Databáze:	MEDLINE
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