To collect real-life data from a large cohort of patients with inherited cerebellar ataxia (CA) in Ireland; assessing implications of CA on self-reported quality of life (QoL) and impairments in work and activities. We aimed to compare responses from a heterogeneous population across groups of all ages, different underlying genetic causes and disease durations.

Inherited CA are heterogeneous progressive neurological conditions associated with significant functional limitations. With little information in the literature, an Irish cohort of individuals with CA were asked to complete a comprehensive survey to evaluate disability and health related quality of life (HRQoL).  

Over 250 anonymous surveys were distributed in clinics, by post and at ataxia meetings nationwide. We report observational descriptive data and compare groups using appropriate statistical tests.

129 individuals (54.3% female) completed this cross-sectional study. 47% were unable to work or had to retire due to ataxia; only 9% were in employment. 75% relied on professional or informal care. HRQoL was significantly worse for all domains in individuals with CA compared with the Irish population normative values (p<0.01). Participants with Friedreich’s ataxia (n=56) demonstrated worse physical functioning then those with undetermined ataxia (n=55). Female gender, earlier symptom onset, current employment, retirement and living in a long term care facility were associated with better outcomes and higher sub-scores in different HRQoL domains, while disease duration correlated with worse physical functioning (p <0.05).