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Force dysmetria in spinocerebellar ataxia 6 correlates with functional capacity.

- Front Hum Neurosci (2015)

Bottom Line: Spinocerebellar ataxia type 6 (SCA6) is a genetic disease that causes pure cerebellar degeneration affecting walking, balance, and coordination.Dysmetria was quantified as the force and time error during goal-directed contractions.We found that SCA6 participants exhibited greater force dysmetria than healthy controls (P < 0.05), and reduced time dysmetria than healthy controls (P < 0.05).

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ABSTRACT
Spinocerebellar ataxia type 6 (SCA6) is a genetic disease that causes pure cerebellar degeneration affecting walking, balance, and coordination. One of the main symptoms of SCA6 is dysmetria. The magnitude of dysmetria and its relation to functional capacity in SCA6 has not been studied. Our purpose was to quantify dysmetria and determine the relation between dysmetria and functional capacity in SCA6. Ten individuals diagnosed and genetically confirmed with SCA6 (63.7 ± 7.02 years) and nine age-matched healthy controls (65.9 ± 8.5 years) performed goal-directed isometric contractions with the ankle joint. Dysmetria was quantified as the force and time error during goal-directed contractions. SCA6 functional capacity was determined by ICARS and SARA clinical assessments. We found that SCA6 participants exhibited greater force dysmetria than healthy controls (P < 0.05), and reduced time dysmetria than healthy controls (P < 0.05). Only force dysmetria was significantly related to SCA6 functional capacity, as measured with ICARS kinetic score (R(2) = 0.63), ICARS total score (R(2) = 0.43), and SARA total score (R(2) = 0.46). Our findings demonstrate that SCA6 exhibit force dysmetria and that force dysmetria is associated to SCA6 functional capacity. Quantifying force and time dysmetria in individuals with SCA6 could provide a more objective evaluation of the functional capacity and disease state in SCA6.

No MeSH data available.


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Force dysmetria and clinical assessment. Force dysmetria was strongly associated with ICARS kinetic score (A; R2 = 0.63), and moderately associated with ICARS Total Score (B; R2 = 0.46) and SARA Total Score (C; R2 = 0.46).
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Figure 5: Force dysmetria and clinical assessment. Force dysmetria was strongly associated with ICARS kinetic score (A; R2 = 0.63), and moderately associated with ICARS Total Score (B; R2 = 0.46) and SARA Total Score (C; R2 = 0.46).

Mentions: The above results suggest that SCA6 exhibit greater force dysmetria but lower time dysmetria than healthy controls. To determine the contribution of force and time dysmetria to the functional capacity of individuals with SCA6, we correlated the force and time errors with the clinical assessment scores. Force dysmetria was positively related with the ICARS kinetic score (R2 = 0.63, P = 0.006; Figure 5A), the ICARS Total score (R2 = 0.43, P = 0.04; Figure 5B), and SARA Total score (R2 = 0.46, P = 0.03; Figure 5C). In contrast, time dysmetria was not significantly related with the ICARS kinetic score (R2 = 0.15, P > 0.2), the ICARS Total score (R2 = 0.12, P > 0.2), and SARA Total score (R2 = 0.2, P > 0.2).


Force dysmetria in spinocerebellar ataxia 6 correlates with functional capacity.

- Front Hum Neurosci (2015)

Force dysmetria and clinical assessment. Force dysmetria was strongly associated with ICARS kinetic score (A; R2 = 0.63), and moderately associated with ICARS Total Score (B; R2 = 0.46) and SARA Total Score (C; R2 = 0.46).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4389656&req=5

Figure 5: Force dysmetria and clinical assessment. Force dysmetria was strongly associated with ICARS kinetic score (A; R2 = 0.63), and moderately associated with ICARS Total Score (B; R2 = 0.46) and SARA Total Score (C; R2 = 0.46).
Mentions: The above results suggest that SCA6 exhibit greater force dysmetria but lower time dysmetria than healthy controls. To determine the contribution of force and time dysmetria to the functional capacity of individuals with SCA6, we correlated the force and time errors with the clinical assessment scores. Force dysmetria was positively related with the ICARS kinetic score (R2 = 0.63, P = 0.006; Figure 5A), the ICARS Total score (R2 = 0.43, P = 0.04; Figure 5B), and SARA Total score (R2 = 0.46, P = 0.03; Figure 5C). In contrast, time dysmetria was not significantly related with the ICARS kinetic score (R2 = 0.15, P > 0.2), the ICARS Total score (R2 = 0.12, P > 0.2), and SARA Total score (R2 = 0.2, P > 0.2).

Bottom Line: Spinocerebellar ataxia type 6 (SCA6) is a genetic disease that causes pure cerebellar degeneration affecting walking, balance, and coordination.Dysmetria was quantified as the force and time error during goal-directed contractions.We found that SCA6 participants exhibited greater force dysmetria than healthy controls (P < 0.05), and reduced time dysmetria than healthy controls (P < 0.05).

View Article: PubMed Central - PubMed

ABSTRACT
Spinocerebellar ataxia type 6 (SCA6) is a genetic disease that causes pure cerebellar degeneration affecting walking, balance, and coordination. One of the main symptoms of SCA6 is dysmetria. The magnitude of dysmetria and its relation to functional capacity in SCA6 has not been studied. Our purpose was to quantify dysmetria and determine the relation between dysmetria and functional capacity in SCA6. Ten individuals diagnosed and genetically confirmed with SCA6 (63.7 ± 7.02 years) and nine age-matched healthy controls (65.9 ± 8.5 years) performed goal-directed isometric contractions with the ankle joint. Dysmetria was quantified as the force and time error during goal-directed contractions. SCA6 functional capacity was determined by ICARS and SARA clinical assessments. We found that SCA6 participants exhibited greater force dysmetria than healthy controls (P < 0.05), and reduced time dysmetria than healthy controls (P < 0.05). Only force dysmetria was significantly related to SCA6 functional capacity, as measured with ICARS kinetic score (R(2) = 0.63), ICARS total score (R(2) = 0.43), and SARA total score (R(2) = 0.46). Our findings demonstrate that SCA6 exhibit force dysmetria and that force dysmetria is associated to SCA6 functional capacity. Quantifying force and time dysmetria in individuals with SCA6 could provide a more objective evaluation of the functional capacity and disease state in SCA6.

No MeSH data available.


Related in: MedlinePlus