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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).

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

Representative end-points of the 50 trials during the goal-directed task for a SCA6 and an age-matched control participant relative to the target. The SCA6 participant exhibited greater force dysmetria than the control participant.
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Figure 2: Representative end-points of the 50 trials during the goal-directed task for a SCA6 and an age-matched control participant relative to the target. The SCA6 participant exhibited greater force dysmetria than the control participant.

Mentions: We quantified force and time dysmetria with the force and time endpoint accuracy of an ankle dorsiflexion goal-directed task. Figure 2 provides a representative result from one SCA6 participant and one healthy control participant. Task acquisition was similar for SCA6 and controls, as it is evident from similar changes in force (Figure 3A; P > 0.2) and time error (Figure 3B; P > 0.2). Overall, SCA6 exhibited greater ankle dorsiflexion force error (Figure 4A; t = 1.74, P < 0.05) and lower time error (Figure 4A; t = -2.27, P < 0.05) than healthy controls.


Force dysmetria in spinocerebellar ataxia 6 correlates with functional capacity.

- Front Hum Neurosci (2015)

Representative end-points of the 50 trials during the goal-directed task for a SCA6 and an age-matched control participant relative to the target. The SCA6 participant exhibited greater force dysmetria than the control participant.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 2: Representative end-points of the 50 trials during the goal-directed task for a SCA6 and an age-matched control participant relative to the target. The SCA6 participant exhibited greater force dysmetria than the control participant.
Mentions: We quantified force and time dysmetria with the force and time endpoint accuracy of an ankle dorsiflexion goal-directed task. Figure 2 provides a representative result from one SCA6 participant and one healthy control participant. Task acquisition was similar for SCA6 and controls, as it is evident from similar changes in force (Figure 3A; P > 0.2) and time error (Figure 3B; P > 0.2). Overall, SCA6 exhibited greater ankle dorsiflexion force error (Figure 4A; t = 1.74, P < 0.05) and lower time error (Figure 4A; t = -2.27, P < 0.05) than healthy controls.

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