Laudiane Reis Santos1, Hélio Afonso Ghizoni Teive2, Francisco Diego Negrão Lopes Neto3, Ana Carolina Brandt de Macedo4, Neliana Maria de Mello5, Marise Bueno Zonta1,5. 1. Universidade Federal do Paraná, Hospital de Clínicas, Programa de Residência Integrada Multiprofissional em Cuidados Hospitalares - Saúde para Adultos e Idosos Curitiba PR, Brasil. 2. Universidade Federal do Paraná, Hospital de Clínicas, Departamento de Medicina Interna, Serviço de Neurologia, Setor de Distúrbios do Movimento, Curitiba PR, Brasil. 3. Universidade Federal do Paraná, Hospital de Clínicas, Serviço de Estatística, Curitiba PR, Brasil. 4. Universidade Federal do Paraná, Departamento de Fisioterapia Preventiva e Reabilitação, Curitiba PR, Brasil. 5. Universidade Federal do Paraná, Serviço de Fisioterapia, Curitiba PR, Brasil.
Abstract
OBJECTIVES: Spinocerebellar ataxia type 10 (SCA10) is characterized by gait ataxia, dysarthria, nystagmus, epilepsy, reduced cognitive ability and depression, which lead to functional loss and behavioral changes. These signs gradually evolve and may interfere with the physical, emotional, and social aspects of quality of life (QoL). To assess the self-perception of quality of life and its association with disease duration, severity of ataxia, balance and functional independence. METHODS: This study focused on the disease duration, ataxia severity (SARA), balance (Berg Balance Scale), functionality (FIM, Lawton IADL) and QoL (SF-36 v.2) of 15 individuals with SCA10. RESULTS: The population sample consisted of eight females and seven males, with a mean age of 43.8 (± 8.2) years, mean age of symptom onset of 33.1 (± 8.9) years and mean disease duration of 9.8 (± 11.2) years. The mean Berg Balance Scale score was 47.2 (± 12), mean SARA score (n = 14) 11.5 (± 7.3), mean Lawton IADL score 20.4 (± 1.8) and mean FIM score 120.3 (± 5.4). Individuals with SCA10 had a greater impairment of QoL in the "role-physical" domain (p = 0.04). The longer the disease duration (p = 0.02), risk of falling (p = 0.04), severity of ataxia (p = 0.00) and functional dependence in activities of daily living (p = 0.03) and instrumental activities of daily living (p = 0.00), the worse the QoL was in the "physical functioning" domain, with a decrease of 1.62 points for each year of disease duration. CONCLUSION: In this sample, the greatest impairment of QoL in individuals with SCA10 was observed in "physical functioning" and "physical role".
OBJECTIVES:Spinocerebellar ataxia type 10 (SCA10) is characterized by gait ataxia, dysarthria, nystagmus, epilepsy, reduced cognitive ability and depression, which lead to functional loss and behavioral changes. These signs gradually evolve and may interfere with the physical, emotional, and social aspects of quality of life (QoL). To assess the self-perception of quality of life and its association with disease duration, severity of ataxia, balance and functional independence. METHODS: This study focused on the disease duration, ataxia severity (SARA), balance (Berg Balance Scale), functionality (FIM, Lawton IADL) and QoL (SF-36 v.2) of 15 individuals with SCA10. RESULTS: The population sample consisted of eight females and seven males, with a mean age of 43.8 (± 8.2) years, mean age of symptom onset of 33.1 (± 8.9) years and mean disease duration of 9.8 (± 11.2) years. The mean Berg Balance Scale score was 47.2 (± 12), mean SARA score (n = 14) 11.5 (± 7.3), mean Lawton IADL score 20.4 (± 1.8) and mean FIM score 120.3 (± 5.4). Individuals with SCA10 had a greater impairment of QoL in the "role-physical" domain (p = 0.04). The longer the disease duration (p = 0.02), risk of falling (p = 0.04), severity of ataxia (p = 0.00) and functional dependence in activities of daily living (p = 0.03) and instrumental activities of daily living (p = 0.00), the worse the QoL was in the "physical functioning" domain, with a decrease of 1.62 points for each year of disease duration. CONCLUSION: In this sample, the greatest impairment of QoL in individuals with SCA10 was observed in "physical functioning" and "physical role".