ABSTRACT
BACKGROUND: Spinocerebellar ataxia (SCA) presents different rates of functional decline depending on the type of ataxia. OBJECTIVE: To compare the progression of disability, imbalance and severity of ataxia in patients with the three most common types of SCA in southern Brazil. METHODS: 126 patients (31-SCA2, 58-SCA3 and 37-SCA10) were stratified into four groups based on disease duration. Progression rates were calculated in each group for ataxia severity (SARA), functioning (FIM-ADL and Lawton-IADL), and balance (Berg Balance Scale). RESULTS: Differences across groups in terms of disease severity revealed a linear pattern of decline in SCA3, with a faster rate over time (p = 0.039) compared to SCA2 and SCA10. The pattern was nonlinear for SCA2 and SCA10, with a twofold faster rate in patients with up to seven years of disease compared to all other periods in SCA10 (p < 0.001) and to the longer follow up period in SCA2 (p = 0.049). Differences across groups regarding worsening of balance scores was significantly faster in SCA3 compared to SCA10 (p = 0.028) and SCA2 (p = 0.028). The rate of loss of independence of ADLs tended to diminish over time in the three types of ataxia and was faster in SCA3. Similarly, the rate for loss of independence (IADLs) was faster in SCA3 compared to SCA2 (p = 0.057) and significantly faster compared to SCA10 (p = 0.028). CONCLUSION: The present findings suggest that the progression of the disease (severity/functioning/balance) varies according to the SCA subtype and the period in disease course. Progression is more linear and aggressive in patients with SCA3.
