2017
DOI: 10.1590/0004-282x20170146
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Impact of disease duration on functional status of patients with spinocerebellar ataxia type 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)… Show more

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Cited by 9 publications
(11 citation statements)
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“…In a study of 16 individuals with SCA2, Amarante et al 29 described how balance, fall risk and ataxia severity evolved over one year of disease. In our study, we found that for every year of disease there was a 1.62-point decrease in the "physical functioning" score, reflecting an increase in disease severity, functional dependence and fall risk.…”
Section: Resultsmentioning
confidence: 99%
“…In a study of 16 individuals with SCA2, Amarante et al 29 described how balance, fall risk and ataxia severity evolved over one year of disease. In our study, we found that for every year of disease there was a 1.62-point decrease in the "physical functioning" score, reflecting an increase in disease severity, functional dependence and fall risk.…”
Section: Resultsmentioning
confidence: 99%
“…Ataxin-2 is a 140-kDa cytoplasmic protein, product of the ATXN2 gene, localized at the rough endoplasmic reticulum. The abnormal ataxin-2 produces a cytosolic protein that is implicated in cytoplasmic RNA-related functions and results in abnormal aggregation, oxidative stress, disturbed cell signaling, dysregulation of calcium homeostasis, abnormal autophagy, and impaired DNA processing, all presumed to be involved in SCA2 molecular pathogenesis (Amarante et al, 2017).…”
Section: Spinocerebellar Ataxia Typementioning
confidence: 99%
“…Other commonly described signs and symptoms are cognitive impairment, peripheral neuropathy with fasciculation, and sleep disorders [5][6][7] . Longer disease duration is associated with greater balance impairment, functional dependence, and ataxia severity in subjects with SCA2 8 .…”
mentioning
confidence: 97%