ABSTRACT Purpose To compare the speech and voice patterns of myasthenia gravis (MG) patients over four years, and correlate the results with clinical aspects of the disease. Methods Data was collected for 4 years. The clinical assessment tools included the Quantitative Myasthenia Gravis (QMG) score, the Myasthenia Gravis Foundation of America (MGFA) clinical classification, and the Myasthenia Gravis Quality of Life 15-item Scale (MG-QoL). To assess speech, the recorded speaking tasks were analyzed acoustically and given auditory-perceptual ratings. Sex (equal distribution) and age (p=0.949) were used as matching criteria in the final sample, which consisted of 10 individuals in the MG group (MGG) and 10 individuals in the control group (CG). Results After 4 years, the MG participants presented stable health status, increased mild and moderate dysarthria (from 40% to 90% of the subjects), and a significant deterioration in the respiration, phonation, and articulation subsystems. The acoustic analysis showed a decline in articulatory patterns (speech rate p=0.047, articulation rate p=0.007, mean syllable duration p=0.007) and vocal quality (increased jitter p=0.022). In the follow-up comparison, there was a significant difference between the phonation variables (shimmer and harmonic-to-noise ratio) of the MGG and CG. Conclusion The MG patients presented a decline in speech over four years and an increase in mild and moderate dysarthria. Despite presenting stable health status, their respiratory, phonatory, and articulatory subsystems worsened. There was no correlation between speech patterns and clinical characteristics of the disease (severity and motor scale).

RESUMO Objetivo Comparar o padrão de fala e voz de indivíduos com Miastenia Gravis (MG) em um intervalo de quatro anos e correlacionar com aspectos clínicos da doença. Método A coleta de dados foi realizada ao longo de 4 anos. A avaliação clínica foi composta pelo Quantitative Myasthenia Gravis Score (QMGS), pela Myasthenia Gravis Foundation of America Classification (MGFA) e pela escala de qualidade de vida para Miastenia Gravis (MG-QOL). A avaliação da fala foi composta por gravação de tarefas, análise perceptivo-auditiva e análise acústica. A amostra final foi composta por 10 indivíduos em MG e 10 indivíduos no grupo controle (GC), pareados por sexo (distribuição igualitária) e idade (p=0,949). Resultados Após 4 anos, os indivíduos com MG apresentaram estabilidade clínica, aumento do diagnóstico de disartria leve e moderada (de 40% para 90% dos sujeitos) e diminuição significativa no desempenho dos subsistemas da fala: respiração, fonação e articulação. Na análise acústica, houve declínio do padrão articulatório (taxa de fala p=0,047, taxa de articulação p=0,007, duração média das sílabas p=0,007) e qualidade vocal (jitter aumentado p=0,022). Houve diferença significativa nas variáveis fonatórias (shimmer e harmonic-to-noise ratio) entre os grupos MG e GC na comparação do seguimento. Conclusão Indivíduos com MG apresentaram declínio no padrão de fala em um intervalo de quatro anos, com aumento no número de disártricos (leve e moderado). Mesmo com a estabilidade da doença, houve piora dos subsistemas respiratório, fonatório e articulatório. Não houve correlação entre o padrão de fala e as características clínicas da doença (gravidade e escala motora).

Abstract Objective: To describe the speech pattern of patients with hereditary Spastic Paraplegia type 4 (SPG4) and correlated it with their clinical data. Methods: Cross-sectional study was carried out in two university hospitals in Brazil. Two groups participated in the study: the case group (n = 28) with a confirmed genetic diagnosis for SPG4 and a control group (n = 17) matched for sex and age. The speech assessment of both groups included: speech task recording, acoustic analysis, and auditory-perceptual analysis. In addition, disease severity was assessed with the Spastic Paraplegia Rating Scale (SPRS). Results: In the auditory-perceptual analysis, 53.5% (n = 15) of individuals with SPG4 were dysarthric, with mild to moderate changes in the subsystems of phonation and articulation. On acoustic analysis, SPG4 subjects’ performances were worse in measurements related to breathing (maximum phonation time) and articulation (speech rate, articulation rate). The articulation variables (speech rate, articulation rate) are related to the age of onset of the first motor symptom. Conclusion: Dysarthria in SPG4 is frequent and mild, and it did not evolve in conjunction with more advanced motor diseases. This data suggest that diagnosed patients should be screened and referred for speech therapy evaluation and those pathophysiological mechanisms of speech involvement may differ from the length-dependent degeneration of the corticospinal tract.

RESUMO Antecedentes: Embora haja predomínio de fraqueza muscular facial na distrofia facioescapuloumeral (FSHD), é escassa a literatura sobre aspectos de fala e deglutição. Objetivo: Investigar os padrões de fala e deglutição na FSHD e correlacioná-los com dados clínicos da doença. Métodos: Estudo transversal. Pacientes com confirmação clínica de FSHD e idade acima de 18 anos foram incluídos e pareados por idade e sexo com controles saudáveis. Foram excluídos indivíduos que apresentassem condições neurológicas que pudessem interferir nos resultados dos testes. Aplicaram-se as seguintes avaliações: fala (análise acústica e perceptivo-auditiva); deglutição, por meio do Northwestern Dysphagia Patient Check Sheet (NDPCS), Eat Assessment Tool (EAT-10), Protocolo de Avaliação para Risco de Disfagia (PARD) e Functional Oral Intake Scale (FOIS); estadiamento da doença, por meio da Gardner-Medwin-Walton scale (GMWS); e qualidade de vida, com o Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36). Resultados de fala e deglutição foram correlacionados com dados clínicos da doença por teste não paramétrico. Resultados: Foram avaliados 13 indivíduos com FSHD e dez controles saudáveis. Houve diferença significativa entre os grupos nas bases motoras fonação e respiração. Na deglutição, dois (15%) indivíduos apresentaram disfagia leve e sete (53,8%), força reduzida da musculatura da face. Esses resultados não foram correlacionados com tempo de doença, idade de início dos sintomas e qualidade de vida. A disfagia esteve relacionada com a gravidade da doença. Conclusões: Pacientes com FSHD apresentaram disartria e disfagia leves. O monitoramento frequente desses sintomas pode ser uma forma importante de proporcionar reabilitação precoce e melhor qualidade de vida.

ABSTRACT 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.

RESUMO Objetivo Identificar os fatores associados à fragilidade em pacientes com doenças neurodegenerativas. Método Estudo transversal, cuja amostra foi composta por 150 pacientes com diagnóstico de doenças neurodegenerativas atendidos em um ambulatório de Fonoaudiologia de um hospital de referência no sul do Brasil. Foi realizada análise secundária exploratória dos prontuários dos pacientes atendidos neste ambulatório entre o período de abril de 2016 e maio de 2019. As informações coletadas foram: sexo, idade, escolaridade, tipo de doença neurodegenerativa, tempo de doença, fragilidade (Edmonton Frail Scale - EFS), deglutição (Northwestern Dysphagia Patient CheckSheet- NDPCS, Eating Assessment Tool-EAT 10) e cognição (Mini-Mental State Examination-MMSE e Montreal Cognitive Assessment-MoCA). As variáveis quantitativas contínuas foram analisadas a partir de média e desvio padrão e as quantitativas categóricas a partir de frequência absoluta e relativa, assim como analisou-se a associação destas com o desfecho pelo teste Qui-Quadrado. As Razões de Prevalência brutas e ajustadas foram avaliadas a partir da Regressão de Poisson com variância robusta. Todos os testes estatísticos foram considerados significativos a um nível de 5%. Resultados Os fatores significativos associados à fragilidade foram à presença de disfagia orofaríngea e desempenho cognitivo alterado. Indivíduos com a fragilidade apresentam maior prevalência de disfagia orofaríngea (RP=1,772(1,094-2,872)), enquanto a cognição alterada está associada a menor prevalência de fragilidade (RP=0,335(0,128-0,873). Conclusão A disfagia orofaríngea pode ser um importante fator clínico preditivo a ser considerado em casos de fragilidade em pacientes com doenças neurodegenerativas.

ABSTRACT Purpose To identify the factors associated with frailty in patients with neurodegenerative diseases. Methods Cross-sectional study, whose sample consisted of 150 patients diagnosed with neurodegenerative diseases seen at a speech-language therapy clinic in a reference hospital in southern Brazil. A secondary exploratory analysis of the medical records of patients treated at this clinic between April 2016 and May 2019 was performed. The information collected was sex, age, education, type of neurodegenerative disease, time of disease, frailty (Edmonton Frail Scale-EFS), swallowing (Northwestern Dysphagia Patient CheckSheet-NDPCS, Eating Assessment Tool-EAT 10), and cognition (Mini-Mental State Examination-MMSE and Montreal Cognitive Assessment-MoCA). Continuous quantitative variables were analyzed using mean and standard deviation and categorical quantitative variables from absolute and relative frequency, as well as their association with the outcome using the Chi-square test. Crude and adjusted Prevalence Ratios were assessed using Poisson regression with robust variance. All statistical tests were considered significant at a level of 5%. Results The significant factors associated with frailty were the presence of oropharyngeal dysphagia and altered cognitive performance. Individuals with frailty have a higher prevalence of oropharyngeal dysphagia (PR= 1.772(1.094-2.872)), while cognition alteration presented a lower prevalence (PR= 0.335(0.128-0.873). Conclusion Oropharyngeal dysphagia can be an important clinical predictive factor for consideration in cases of frailty in patients with neurodegenerative diseases.

RESUMO Embora seja uma doença predominantemente muscular, alterações no sistema nervoso central em pacientes com Distrofia Facioescapuloumeral (FSHD) têm sido descritas na literatura. Objetivo: Caracterizar o perfil cognitivo de pacientes com FSHD e correlacionar as alterações encontradas com variáveis clínicas e qualidade de vida. Métodos: Estudo transversal, caso-controle que avaliou pacientes com FSHD por meio de uma série de avaliações cognitivas (Mini Exame do Estado Mental — MEEM; Montreal Cognitive Assessment — MoCA; fluência verbal com restrição fonológica — FAS; fluência verbal categórica — FAS-cat; Trail Making Test — TMT; e Rey’s Verbal Auditory Learning Test — RAVLT); uma escala de gravidade neurológica (Gardner-Medwin-Walton — GMWS); e um questionário (Medical Outcomes Study 36-Item Short-Form Health Survey — SF-36). Resultados: A amostra foi composta por 13 indivíduos com FSHD e 26 controles saudáveis, pareados por sexo e idade. A análise comparativa entre os grupos FSHD e controle mostrou diferenças significativas no desempenho cognitivo dos testes MEEM, TMT A e A7 (p≤0.05) e no MoCA (p≤0,001). Verificou-se uma correlação positiva com a idade de início dos sintomas e o prejuízo na memória de longo prazo (r=-0,593, p=0,033). Em relação à qualidade de vida, observou-se uma correlação entre o domínio de limitação emocional e os testes MEEM (r=0,657, p=0,015), TMT A (r=-0,601, p=0,030) e A7 (r=0,617, p=0,025). Conclusões: Pacientes com FSHD apresentaram alterações leves na realização de tarefas que envolvem as funções de atenção, planejamento e memória de longo prazo. Essas alterações não tiveram associação com o tempo de doença nem com sua gravidade neurológica.

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.

RESUMO. Investigamos o desempenho cognitivo de pacientes com miastenia gravis (MG) por meio de um estudo transversal. Aplicou-se uma bateria de avaliações cognitivas e questionários de autopercepção sobre qualidade de vida (QV), sono e depressão. A amostra foi composta por 39 pacientes com diagnóstico de MG. Os escores mostraram predominância de comprometimento cognitivo no teste de rastreio Montreal Cognitive Assessment (MoCA) (66,7%) e nas tarefas de memória imediata (59,0%) e recente (56,4%). Entretanto, após a análise de regressão de Poisson com variância robusta, verificou-se que os pacientes diagnosticados com depressão apresentaram uma razão de prevalência (RP)=1.887 (IC 1.166‒3.054) para escores mais baixos no MoCA, RP=9.533 (IC 1.600‒56.788) nos testes de fluência verbal fonêmica e RP=12.426 (IC 2.177‒70.931) no teste de fluência verbal semântica. Além disso, uma associação entre pior desempenho nas tarefas de memória de retenção de curto prazo nos pacientes em uso de glucocorticoides (GC) e com os escores do Beck Depression Inventory indicando depressão, com RP=11.227 (IC 1.736‒72.604) e RP=0.35 (IC 0.13‒0.904), respectivamente. Não foi encontrada correlação entre o questionário de QV e o desempenho em testes cognitivos. Sendo assim, conclui-se que foi observado pior desempenho em tarefas de memória e funções executivas em pacientes com MG. Estes não estão associados ao tempo e à gravidade da doença. No entanto, uma taxa de prevalência significativa foi encontrada para pior desempenho da memória em pacientes diagnosticados com depressão e naqueles em uso de glucocorticoides.

ABSTRACT. We investigated the cognitive performance of patients with Myasthenia Gravis (MG) through a cross-sectional study. A battery of cognitive assessments and self-report questionnaires regarding quality of life (QoL), sleep, and depression were applied. The sample consisted of 39 patients diagnosed with MG. The scores showed a predominance of cognitive impairment in the Montreal Cognitive Assessment screening test (MoCA) (66.7%) and in the immediate (59.0%) and recent memory (56.4%) tests. However, after the Poisson regression analysis with robust variance, it was found that patients diagnosed with depression had a prevalence ratio (PR) of 1,887 (CI 1,166‒3,054) for lower MoCA scores, PR=9,533 (CI 1,600‒56,788) for poorer phonemic verbal fluency scores, and PR=12,426 (CI 2,177‒70,931) for the Semantic Verbal Fluency test. Moreover, concerning a decline in short-term memory retention, patients using glucocorticosteroids (GC) and with Beck Depression Inventory scores indicating depression showed PR=11,227 (CI 1,736‒72,604) and PR=0.35 (CI 0.13‒0.904), respectively. No correlation was found between the QoL questionnaire and performance in cognitive tests. We found worse performance in tasks of memory and executive functions in MG patients. These are not associated with the length and severity of the disease. However, a significant prevalence ratio was found for poorer memory performance in patients diagnosed with depression and in those using GC.

RESUMO As paraparesias espásticas hereditárias (PEH) são um grupo de doenças genéticas caracterizado por espasticidade dos membros inferiores com ou sem características neurológicas adicionais. A disfunção da deglutição é pouco estudada nas PEH e sua presença pode levar a complicações respiratórias e nutricionais significativas. Objetivo: O objetivo deste estudo foi avaliar a frequência e a caracterização clínica da disfagia em diferentes tipos de PEH. Métodos: Foi realizado um estudo transversal em dois centros. Os pacientes com PEH confirmados geneticamente foram avaliados pelo Northwestern Dysphagia Patient Check Sheet e pela Escala Funcional de Ingestão Oral. Além disso, a autopercepção da disfagia foi avaliada pelo Eat Assessment Tool-10 e pelo Swallowing Disturbance Questionnaire. Resultados: Trinta e seis pacientes com paraplegia espástica tipo 4 (SPG4), cinco com SPG11, quatro com SPG5, quatro com xantomatose cerebrotendinosa (CTX), três com SPG7 e dois com SPG3A foram avaliados. Disfagia orofaríngea leve a moderada estava presente em 3/5 (60%) dos pacientes com SPG11 e 2/4 (50%) dos pacientes com CTX. Um único SPG4 (2%) e um único SPG7 (33%) apresentaram disfagia orofaríngea leve. Todos os outros pacientes avaliados apresentaram deglutição normal ou funcional. Conclusão: Disfagia orofaríngea clinicamente significativa estava presente apenas nas formas complicadas de PEH. A SPG11 e CTX apresentaram maiores riscos de disfagia, sugerindo que a avaliação da deglutição deve fazer parte do manejo dos pacientes com essas condições.

ABSTRACT Hereditary spastic paraplegias (HSP) are a group of genetic diseases characterized by lower limb spasticity with or without additional neurological features. Swallowing dysfunction is poorly studied in HSP and its presence can lead to significant respiratory and nutritional complications. Objectives: The aim of this study was to evaluate the frequency and clinical characteristics of dysphagia in different types of HSP. Methods: A two-center cross-sectional prevalence study was performed. Genetically confirmed HSP patients were evaluated using the Northwestern Dysphagia Patient Check Sheet and the Functional Oral Intake Scale. In addition, self-perception of dysphagia was assessed by the Eat Assessment Tool-10 and the Swallowing Disturbance Questionnaire. Results: Thirty-six patients with spastic paraplegia type 4 (SPG4), five with SPG11, four with SPG5, four with cerebrotendinous xanthomatosis (CTX), three with SPG7, and two with SPG3A were evaluated. Mild to moderate oropharyngeal dysphagia was present in 3/5 (60%) of SPG11 and 2/4 (50%) of CTX patients. A single SPG4 (2%) and a single SPG7 (33%) patient had mild oropharyngeal dysphagia. All other evaluated patients presented with normal or functional swallowing. Conclusions: Clinically significant oropharyngeal dysphagia was only present in complicated forms of HSP Patients with SPG11 and CTX had the highest risks for dysphagia, suggesting that surveillance of swallowing function should be part of the management of patients with these disorders.

Abstract Introduction Deep brain stimulation (DBS) is an established treatment for Parkinson’s disease (PD). However, there is little evidence regarding the effect of DBS on dysphagia. Objective To assess the swallowing and quality of life of individuals with PD before and after DBS surgery. Methods Our sample consisted of people who had undergone DBS surgery in a referral hospital in the state of Rio Grande do Sul, Brazil. The inclusion criteria were a diagnosis of PD and having undergone DBS surgery. A cognitive screening, through a questionnaire about depression and quality of life, was conducted. Evaluations of each patient’s swallowing were performed before and after surgery. The assessment consisted of anamnesis, clinical assessment, the Functional Oral Intake Scale, clinical evaluation of swallowing, and the Hoehn and Yahr scale. Results The sample included 10 individuals, all male, with a mean age of 57.3 years (±4.7), a mean disease duration of 13.0 years (±2.4), and mean level education of 8.1 years (±4.0). In the clinical evaluation of the swallowing, a significant improvement after DBS was not observed. However, little changes in the signs and symptoms of dysphagia that had a positive impact on the quality of life were observed. Furthermore, there was no relation between the patients’ motor subtype and swallowing pre- and post-DBS. Conclusion There was an improvement in the quality of life of the patients after DBS. However, the improvement in the clinical signs and symptoms of dysphagia did not cause an overall improvement in the swallowing function.

RESUMO Introdução A doença de Parkinson (DP) é a segunda doença neurodegenerativa mais prevalente no mundo, com predomínio de sintomas motores e não motores. Dentre estes, destaca-se a disfagia. Objetivo Realizar a revisão sistemática da literatura que trata das avaliações clínicas não instrumentais disponíveis para rastreio e avaliação da disfagia em indivíduos com DP. Estratégia de pesquisa Para a seleção dos estudos, foram utilizados os descritores Parkinson disease, swallowing, dysphagia, deglutition disorders, questionnaire, health surveys, evaluation, screening e assessment, em combinações variadas, nas seguintes bases de dados: PubMed, Biblioteca Cochrane e SciELO. Critérios de seleção Artigos em inglês, português e espanhol, publicados no período de janeiro de 2006 a julho de 2016, cuja abordagem metodológica atendesse ao objetivo desta revisão. Dois revisores independentes analisaram os artigos, a fim de verificar a elegibilidade. Quando houve discordância, o consenso foi alcançado pela avaliação de um juiz que não conhecia as avaliações anteriores. Resultados Foram encontrados 846 artigos. Após consideração dos critérios de inclusão/exclusão e análise do juíz, apenas 4 estudos foram analisados, nos quais foram utilizados 4 instrumentos diferentes, todos eles questionários de autopercepção da disfagia. Não houve nenhum instrumento que tivesse realizado avaliação clínica da disfagia com oferta de alimentos. Conclusão Verificou-se que não existem instrumentos de rastreio e de avaliação clínica da disfagia em paciente com DP que utilizem oferta de alimento e não somente auto-percepção do paciente, no período que compreendeu o levantamento bibliográfico deste estudo.

ABSTRACT Introduction Parkinson’s disease (PD) is the second most prevalent neurodegenerative disease in the world, with a predominance of motor and non-motor symptoms. Among these, dysphagia stands out. Purpose Systematically review the non-instrumental clinical evaluations available for the screening and assessment of dysphagia in individuals with PD. Research strategy For the selection of the studies, we used the descriptors: Parkinson disease, swallowing, dysphagia, deglutition disorders, questionnaire, health surveys, evaluation, screening, and evaluation, in a variety of combinations, aiming at a greater number of studies. The databases were PubMed, Cochrane Library, and SciELO. Selection criteria Articles published in English, Portuguese, and Spanish that were published between January 2006 and July 2016 were selected, whose methodological approach met the objective of this review. A descriptive analysis was performed. Two independent reviewers reviewed the articles in order to verify the eligibility. When there was disagreement, a consensus was reached by the evaluation of a judge who did not know the previous evaluations. Results Eight hundred forty-six articles were founded. After considering the inclusion/exclusion criteria and the judge’s analysis, only four studies were analyzed, which were four different instruments, and all of the instruments were self-perception questionnaires of dysphagia. There was no instrument that performed a clinical evaluation of dysphagia with the food supply. Conclusion It has been verified there are no instruments for the screening and clinical evaluation of dysphagia in patients with PD who use a food supply and not only the self-perception of the patient in the period that included the bibliographic survey of this study.

RESUMO Embasamento: As evidências apontam a ocorrência de comprometimento cognitivo em todas as fases da doença de Parkinson (DP), sendo este um sintoma não motor frequente e incapacitante, devido ao alto impacto na qualidade de vida e mortalidade dos pacientes. Objetivo: correlacionar a performance cognitiva com qualidade de vida na DP. Métodos: A amostra foi oriunda de um Ambulatório de Distúrbios do Movimento de um hospital referência de Porto Alegre. Os critérios de inclusão utilizados foram ter diagnóstico de DP e consentir em participar do estudo. Exclui-se pacientes com outras patologias neurológicas ou pacientes com estimulação cerebral profunda. A avaliação foi composta por uma anamnese, bateria de testes cognitivos (composta por oito testes), questionários sobre qualidade de vida (PDQ-39) e depressão (BDI). Resultados: A amostra foi composta por 85 indivíduos com DP, com média de idade de 62,9 anos (±10,7), média do tempo de doença de 10,4 anos (±5,7) e média de escolaridade de 7,4 anos (±4,3). Verificou-se relação significativa entre escore total do PDQ com todos os testes cognitivos, demonstrando que pior performance cognitiva está relacionada com pior qualidade de vida. Além disso, foi observado correlação significativa entre os testes cognitivos com depressão, H&Y, escolaridade e idade. Conclusão: Pode-se concluir que na presente amostra indivíduos com DP apresentaram correlação entre pior qualidade de vida com pior desempenho cognitivo. Isto também foi observado com estágio avançado da doença, idade avançada, baixa escolaridade e depressão.

ABSTRACT Background: Evidence points to the occurrence of cognitive impairment in all stages of PD, constituting a frequent and debilitating symptom, due to high impact on quality of life and mortality of patients. Objective: To correlate cognitive performance with quality of life in PD. Methods: The sample was drawn from a Movement Disorders Clinic of a reference hospital in Porto Alegre. Inclusion criteria were: PD diagnosis, according to the United Kingdom Parkinson's Disease Society Brain Bank criteria for idiopathic PD (Hughes et al. 1992) and patient consent to participate. Patients with other neurological pathologies and those submitted to deep brain stimulation were excluded. The evaluation consisted of a cognitive testing battery (composed of eight tests for assessing cognitive performance), and a questionnaire on quality of life (PDQ-39) and depression (BDI). Results: The sample comprised 85 individuals with PD, with a mean age of 62.9 years (±10.7), mean disease duration of 10.4 years (±5.7), and mean educational level of four years (±4.3). There was a significant relationship between total score on the PDQ and all cognitive tests, showing that poor cognitive performance was correlated with poor quality of life. Moreover, a significant correlation was observed between cognitive tests and depression, H&Y, education level, and age. Conclusion: It may be concluded that the individuals with PD in this sample showed a correlation between poorer quality of life and worse cognitive performance. Poor performance was also correlated with more advanced stage, older age, low level of education and depression.

Abstract Introduction Dysphagia is a common symptom in Parkinson's disease (PD) and it has been associated with poor quality of life (QoL), anxiety, depression. Objective The aim of this study was to evaluate the quality of life in individuals with PD before and after SLP therapy. Methods The program consisted of four individual therapy sessions. Each session comprised guidelines regarding food and postural maneuvers (chin down). The Quality of Life in Swallowing Disorders (SWAL-QOL) questionnaire was applied before and after therapy. Results The sample comprised of 10 individuals (8men), with amean (SD) age of 62.2 (11.3) years, mean educational attainment of 7.5 (4.3) years, andmean disease duration of 10.7 (4.7) years. Thirty percent of patients were Hoehn and Yahr (H&Y) stage 2, 50% were H&Y stage 3, and 20% were H&Y stage 4. Mean scores for all SWAL-QOL domains increased after the intervention period, with significant pre- to post-therapy differences in total score (p = 0.033) and domain 4 (symptom frequency) (p = 0.025). There was also a bias significance for domain 5 (food selection) (p = 0.095). Conclusion Patients exhibited improvement in swallowing-related quality of life after a SLP therapy program. The earlier in the course of PD, greater the improvement observed after therapy.

RESUMO Objetivo: traduzir e adaptar culturalmente para o português brasileiro o Swallowing disturbance questionnaire (SDQ) para detecção de risco de disfagia em indivíduos com Doença de Parkinson (DP). Métodos: realizou-se tradução para o português, retradução para o inglês, análise de confiabilidade do instrumento, sendo a versão final aplicada aos participantes e realizado o reteste após duas semanas. Selecionou-se os participantes em um ambulatório de distúrbios do movimento de um hospital de referência no Rio Grande do Sul. Incluiu-se indivíduos com diagnóstico de DP. Excluiu-se indivíduos com alteração de linguagem ou audição que impossibilitasse a compreensão do questionário e com diagnóstico de outras doenças neurológicas. Resultados: a amostra foi composta por 23 indivíduos. Os participantes responderam a todas as questões. Não houve eliminação de nenhuma questão. Na análise da confiabilidade teste-reteste o coeficiente de correlação intraclasse do escore final nos dois momentos foi de 0,912 com p<0,001 (95%IC=0,792-0,963), demonstrando que os dados são altamente homogêneos. Na análise por questão, não houve diferença significante entre os dois momentos de aplicação. O valor de α de Cronbach do instrumento foi de 0,63. Conclusão: houve equivalência cultural do SDQ para o português brasileiro, com boa confiabilidade interna do instrumento.

ABSTRACT Purpose: to translate and culturally adapt to Brazilian Portuguese Swallowing the disturbance questionnaire (SDQ) for dysphagia detection risk in individuals with Parkinson's disease (PD). Methods: this was translated into Portuguese, back-translation into English, instrument reliability analysis, the final version applied to participants and conducted retesting after two weeks. It was selected participants at a clinic for movement disorders of a reference hospital in Rio Grande do Sul. It was included individuals diagnosed with PD. It was excluded individuals with impaired speech or hearing that prevented the understanding of the questionnaire and diagnosis of other neurological diseases. Results: the sample consisted of 23 individuals. The participants answered all questions. There was no question of disposal. In the analysis of test-retest reliability intraclass correlation coefficient of the final score in the two periods was 0.912 with p <0.001 (95% CI = 0.792 to 0.963), demonstrating that the data are highly homogeneous. In the analysis by question, there was no significant difference between the two application times. The value of α Cronbach instrument was 0.63. Conclusion: there was cultural equivalence of the SDQ for Brazilian Portuguese, with good internal reliability of the instrument.