RESUMO Objetivo Determinar efetividade da terapia de sensibilidade oral em idosos institucionalizados com demência grave. Métodos Uma série de oito casos residentes em instituição de longa permanência. Foram incluídos idosos com diagnóstico de demência grave e disfagia grave, com consentimento da família. Foram excluídos idosos em atendimento fonoaudiológico e com alimentação somente por via alternativa. A divisão entre grupo experimental e grupo controle foi por sorteio. A avaliação pré e pós terapia, realizada por uma fonoaudióloga cega para a intervenção, utilizou protocolo Northwestern Patient Dysphagia Check Sheet (NDPCS), a escala Clinical Dementia Rating (CDR) e a Functional Oral Intake Scale (FOIS). Durante a oferta a velocidade da deglutição e o número de deglutições (pastoso e líquido) foram avaliados. Os participantes do grupo experimental foram submetidos a cinco sessões consecutivas de estimulação tátil-térmica por 30 minutos. Resultados Oito participantes, com idade entre 68 e 98 anos, fizeram parte da amostra seis mulheres e dois homens. No grupo experimental não observou-se modificação na velocidade da deglutição bem como no número de deglutições nas consistências pastosa e líquida. Não foram encontradas diferenças significativas para os itens 23 a 28 do instrumento NDPCS nas duas consistências, bem como não houve modificação na escala FOIS. Também não houve diferenças estatisticamente significativas entre o grupo experimental e o grupo controle para essas duas consistências. Conclusão A estimulação tátil-térmica não modificou o padrão de deglutição em idosos institucionalizados com demência grave.
Background: Although facial muscle weakness is common in patients with Facioscapulohumeral Muscular Dystrophy (FSHD), the literature is scarce on the speech and swallowing aspects. Objective: To investigate speech and swallowing patterns in FSHD and assess the correlation with clinical data. Methods: A cross-sectional study was conducted. Patients with clinical confirmation of FSHD and aged above 18 years were included and paired with healthy control individuals by age and gender. Individuals who had neurological conditions that could interfere with test results were excluded. The following assessments were applied: speech tests (acoustic and auditory-perceptual analysis); swallowing tests with the Northwestern Dysphagia Patient Check Sheet (NDPCS), the Eat Assessment Tool (EAT-10), the Speech Therapy Protocol for Dysphagia Risk (PARD), and the Functional Oral Intake Scale (FOIS); disease staging using the modified Gardner-Medwin-Walton scale (GMWS); and quality of life with the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36). The correlation between test results and clinical data was verified by non-parametric statistics. Results: Thirteen individuals with FSHD and 10 healthy controls were evaluated. The groups presented significant differences in the motor bases of phonation and breathing. Regarding swallowing, two (15%) individuals presented mild dysphagia and seven (53.8%) showed reduced facial muscles strength. These results were not correlated with duration of the disease, age at symptoms onset, and quality of life. Dysphagia was related to worsening disease severity. Conclusions: FSHD patients presented mild dysarthria and dysphagia. Frequent monitoring of these symptoms could be an important way to provide early rehabilitation and better quality of life.
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.
Background: Spinocerebellar ataxia type 3/Machado-Joseph Disease (SCA3/MJD) is a genetic disorder characterized by ataxic manifestations. Although dysarthria is the main symptom, validated assessments of speech and voice are lacking. Objectives: We aimed to evaluate reliability of auditory-perceptual and acoustic assessments, collected in-person and by telephone calls, and to raise data on their external validity. Methods: Symptomatic individuals were studied. Arm 1 studied test-retest reliability of in-person speech assessments; arm 2 studied reliability of in-person and remote evaluations. Friedreich's Ataxia Rating Scale (FARS)-adl, demographic, and molecular data were also obtained. The minimum kappa for the five perceptual variables under study to be considered reliable was set at 0.8. The minimum intraclass correlation coefficients (ICC) for the 45 acoustic-instrumental assessments under study to be considered reliable was 0.70, for a p <0.05. Results: 17 and 20 subjects were included in arms 1 and 2, respectively. All five auditory-perceptual assessments and 30 acoustic-instrumental assessments were considered reliable for the standard in-person use. Four auditory-perceptual and four acoustic assessments have also achieved reliability for use in-person and/or by telephone. Three auditory-perceptual assessments (subsystems: phonation, respiration, and resonance) and two acoustic-instrumental assessments (subsystems: respiration and articulation) correlated with age and disease duration; phonation also correlated FARS-adl. Discussion: A substantial number of assessments of speech were considered reliable for the standard in-person use in SCA3/MJD. Moreover, eight of them were also reliable when used either in-person or remotely. Our results support their use in future studies on this disease.
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