Cognitive profile of patients with facioscapulohumeral muscular dystrophy
ABSTRACT 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.
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Academia Brasileira de Neurologia, Departamento de Neurologia Cognitiva e Envelhecimento
2021
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oai:scielo:S1980-576420210004005412021-12-01Cognitive profile of patients with facioscapulohumeral muscular dystrophySantos,Vanessa Brzoskowski dosSaute,Jonas Alex MoralesJacinto-Scudeiro,Laís AlvesAyres,AnneliseRech,Rafaela SoaresOliveira,Alcyr Alves deOlchik,Maira Rozenfeld muscular dystrophies neuromuscular diseases cognition quality of life ABSTRACT 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.info:eu-repo/semantics/openAccessAcademia Brasileira de Neurologia, Departamento de Neurologia Cognitiva e EnvelhecimentoDementia & Neuropsychologia v.15 n.4 20212021-12-01info:eu-repo/semantics/articletext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1980-57642021000400541en10.1590/1980-57642021dn15-040015 |
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Santos,Vanessa Brzoskowski dos Saute,Jonas Alex Morales Jacinto-Scudeiro,Laís Alves Ayres,Annelise Rech,Rafaela Soares Oliveira,Alcyr Alves de Olchik,Maira Rozenfeld |
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Santos,Vanessa Brzoskowski dos Saute,Jonas Alex Morales Jacinto-Scudeiro,Laís Alves Ayres,Annelise Rech,Rafaela Soares Oliveira,Alcyr Alves de Olchik,Maira Rozenfeld Cognitive profile of patients with facioscapulohumeral muscular dystrophy |
author_facet |
Santos,Vanessa Brzoskowski dos Saute,Jonas Alex Morales Jacinto-Scudeiro,Laís Alves Ayres,Annelise Rech,Rafaela Soares Oliveira,Alcyr Alves de Olchik,Maira Rozenfeld |
author_sort |
Santos,Vanessa Brzoskowski dos |
title |
Cognitive profile of patients with facioscapulohumeral muscular dystrophy |
title_short |
Cognitive profile of patients with facioscapulohumeral muscular dystrophy |
title_full |
Cognitive profile of patients with facioscapulohumeral muscular dystrophy |
title_fullStr |
Cognitive profile of patients with facioscapulohumeral muscular dystrophy |
title_full_unstemmed |
Cognitive profile of patients with facioscapulohumeral muscular dystrophy |
title_sort |
cognitive profile of patients with facioscapulohumeral muscular dystrophy |
description |
ABSTRACT 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. |
publisher |
Academia Brasileira de Neurologia, Departamento de Neurologia Cognitiva e Envelhecimento |
publishDate |
2021 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1980-57642021000400541 |
work_keys_str_mv |
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