Objective: To compare the differences in the evolution in terms of severity, functional independence and balance in patients with SCAs type 3 and 10

Background: Spinocerebellar ataxias (SCAs) types 3 and 10 represent the most common SCAs in the South of Brazil.

Methods: We selected patients from the Ataxia Outpatient Clinic of the Movement Disorders Unit of Hospital de Clínicas, Federal University of Paraná, who had been diagnosed with either SCA 3 or 10 between January 2013 and May 2016. We evaluated duration and severity scores of cerebellar ataxia, with the Scale for the Assessment and Rating of Ataxia (SARA), independence with the Functional Independence Measure (FIM) and balance with the Berg Balance Scale (BBS).

Results: We evaluated 35 patients with SCA type 10 and 60 patients with SCA type 3. The mean age in SCA10 and SCA3 in our sample was 45 ± 9.5; 43.8 ± 12.3; the mean age of onset of symptoms was 33.88 ± 8.3; 34.8 ± 10.7 and the mean duration of disease was 11.57 ± 9.00 and 7.5 ± 4.9 years, respectively. Even though the severity of symptoms as measured with the SARA is similar in the two SCAs in the first five years of disease, there was marked worsening after this period in SCA3 if compared to SCA10 (p <0.0001). Sub-items of the SARA assessing the severity gait (p<0.0003), posture (p<0.0021) and sitting (p<0.0008) showed the greatest difference between the two SCAs. Balance (p<0.0054) and functional independence (p<0.0008) were relatively preserved in SCA10 as they lasted longer, as well as the items of the MIF related to motor score (p<0.0004) and cognitive (p<0.0173).

Conclusions: Patients with SCA10 have a slower progression of the disease if compared to SCA3, maintaining functional independence and balance over a longer period.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/severity-assessment-functional-capacity-and-balance-in-patients-with-spinocerebellar-ataxias-scas-comparison-between-types-3-and-10/