Objective: To evaluate quality of life (QoL) of individuals with Spinocerebellar Ataxia Type 10 (SCA10) and investigate whether there is an association between self-perception and disease duration, severity of ataxia, balance and functional dependence.

Background: SCA10 is a progressive disorder that leads to functional loss that may interfere with physical, emotional, and social aspects of QoL.

Methods: Prospective, descriptive and cross-sectional study considering disease duration and the scores for: severity of ataxia (Scale for the Assessment and Rating of Ataxia – SARA), balance/fall risk (Berg Balance Scale – BBS), independence for daily (ADL) and Instrumental (IADL) activities (Functional Independence Measure – FIM; Lawton IADL Scale) and QoL (Medical Outcomes Study 36-Item Short-Form Health Survey Version 2 – SF-36 v.2) of individuals with SCA10.

Results: Fifteen individuals with SCA10 were evaluated, 8 females, with a mean age of 43.8 (±8.2) years, mean age at onset of symptoms was 33.1 (±8.9) years and mean duration of disease 9.8 (±11.2) years. Most of the study population was independent in ADL and partially dependent in IADL, had a low fall risk (independent balance) and mild ataxia. Demographic, clinical, laboratory and functional data for each individual are shown in Table 1. Individuals with SCA10 had greater impairment of QoL in the “Role-Physical” domain (p=0.04) (Table 2).The greater 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 (ADL) (p=0.03) and instrumental activities of daily living (IADL) (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.

Conclusions: The greatest impairment in QoL in this sample of individuals with SCA10 was observed in the “Role-Physical” and “Physical Functioning” domains.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/influence-of-disease-progression-on-the-quality-of-life-of-individuals-with-spinocerebellar-ataxia-type-10/