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Impact of disease duration on functional status of patients with spinocerebellar ataxia type 2

Repercussões do tempo de evolução sobre o perfil funcional de pacientes com ataxia espinocerebelar do tipo 2

ABSTRACT

Objective:

To correlate disease duration in spinocerebellar ataxia type 2 (SCA2) with disease severity, balance and functionality.

Method:

Sixteen SCA2 patients were analyzed for: disease duration, disease severity (SARA score), balance (Berg balance scale score) and functionality (FIM and Lawton scores).

Results:

Greater severity was correlated with worse functionality (Lawton: r = −0.0561, FIM: r = −0.6402) and balance (r = −0.7188). Longer disease duration was correlated with greater severity (p = 0.0002) and reduced functionality (FIM: p = 0.005; Lawton: p = 0.0402) and balance (p = 0.0036). A year increase in disease duration corresponded to a 0.8-point increase on the SARA scale, a 1.38-point decrease in FIM score, a 2.30-point decrease on the Berg balance scale and a 0.45-point decrease on the Lawton scale.

Conclusion:

Longer disease duration in this series of SCA2 patients was correlated with greater disease severity, worse balance and greater functional dependency.

Keywords:
spinocerebellar ataxias; severity of illness index; postural balance

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