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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

Goal-directed task. (A) Subjects were seated with the hip, knee, and ankle joint at 90°. The left foot was restrained in an ankle device. Subjects performed a goal-directed ankle dorsiflexion contraction with the left foot. (B) Force and time-to peak force errors were normalized to the targeted force and time.
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Figure 1: Goal-directed task. (A) Subjects were seated with the hip, knee, and ankle joint at 90°. The left foot was restrained in an ankle device. Subjects performed a goal-directed ankle dorsiflexion contraction with the left foot. (B) Force and time-to peak force errors were normalized to the targeted force and time.

Mentions: For the isometric goal-directed task, the left hip joint was flexed to ∼90° and abducted by ∼10°, and the knee was flexed to ∼90°. The left foot rested on a customized foot device with an adjustable foot plate and was secured by straps over the metatarsals to ensure an isolated dorsiflexion of the ankle (Figure 1A). The initial ankle position was ∼90° of ankle dorsiflexion. We chose the left leg for this study because it was the non-dominant limb and thus the task would be more difficult for the participants compared with the dominant limb (Christou et al., 2007).


Force dysmetria in spinocerebellar ataxia 6 correlates with functional capacity.

- Front Hum Neurosci (2015)

Goal-directed task. (A) Subjects were seated with the hip, knee, and ankle joint at 90°. The left foot was restrained in an ankle device. Subjects performed a goal-directed ankle dorsiflexion contraction with the left foot. (B) Force and time-to peak force errors were normalized to the targeted force and time.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 1: Goal-directed task. (A) Subjects were seated with the hip, knee, and ankle joint at 90°. The left foot was restrained in an ankle device. Subjects performed a goal-directed ankle dorsiflexion contraction with the left foot. (B) Force and time-to peak force errors were normalized to the targeted force and time.
Mentions: For the isometric goal-directed task, the left hip joint was flexed to ∼90° and abducted by ∼10°, and the knee was flexed to ∼90°. The left foot rested on a customized foot device with an adjustable foot plate and was secured by straps over the metatarsals to ensure an isolated dorsiflexion of the ankle (Figure 1A). The initial ankle position was ∼90° of ankle dorsiflexion. We chose the left leg for this study because it was the non-dominant limb and thus the task would be more difficult for the participants compared with the dominant limb (Christou et al., 2007).

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