Prevalence of oropharyngeal dysphagia in hereditary spastic paraplegias

Autor: Daniela Burguêz, Carelis González-Salazar, Annelise Ayres, Jonas Alex Morales Saute, Márcia Polese-Bonatto, Marcondes Cavalcante França Junior, Gustavo Dariva Machado, Marina Siebert, Laís Alves Jacinto-Scudeiro, Maira Rozenfeld Olchik
Jazyk: angličtina
Rok vydání: 2020
Předmět:
Male
Spastic paraplegia
hereditary

Cross-sectional study
Deglutição
Gastroenterology
Severity of Illness Index
0302 clinical medicine
Risk Factors
Surveys and Questionnaires
Spastic
Prevalence
Medicine
deglutition
deglutição
xantomatose cerebrotendinosa

0303 health sciences
030305 genetics & heredity
Xanthomatosis
Cerebrotendinous

Middle Aged
Dysphagia
transtornos da deglutição
Neurology
Female
medicine.symptom
Paraplegia
Paraplegia espástica hereditária
Brazil
RC321-571
Adult
medicine.medical_specialty
Neurosciences. Biological psychiatry. Neuropsychiatry
Cerebrotendinous Xanthomatosis
03 medical and health sciences
Transtornos de deglutição
Age Distribution
Swallowing
Internal medicine
Severity of illness
otorhinolaryngologic diseases
Humans
Sex Distribution
Aged
xanthomatosis
cerebrotendinous

business.industry
deglutition disorders
medicine.disease
Deglutition
Cross-Sectional Studies
Neurology (clinical)
business
Deglutition Disorders
030217 neurology & neurosurgery
Xantomatose cerebrotendinosa
Oropharyngeal dysphagia
Zdroj: Arquivos de Neuro-Psiquiatria, Vol 77, Iss 12, Pp 843-847 (2020)
Repositório Institucional da UFRGS
Universidade Federal do Rio Grande do Sul (UFRGS)
instacron:UFRGS
Arquivos de Neuro-Psiquiatria, Volume: 77, Issue: 12, Pages: 843-847, Published: 10 JAN 2020
Arquivos de Neuro-Psiquiatria v.77 n.12 2019
Arquivos de neuro-psiquiatria
Academia Brasileira de Neurologia
instacron:ABNEURO
ISSN: 1678-4227
Popis: 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. 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.
Databáze: OpenAIRE