Temporal but not spatial dysmetria relates to disease severity in FA

Author:

Corti Manuela12,Casamento-Moran Agostina3,Delmas Stefan3,Bracksieck Samantha3,Bowman Jessica1,Meyer Blake1,Norman Samantha1,Subramony Sub2,Christou Evangelos A.3

Affiliation:

1. College of Medicine, Department of Pediatrics, University of Florida, Gainesville, Florida

2. College of Medicine, Department of Neurology, University of Florida, Gainesville, Florida

3. College of Health and Human Performance, Department of Applied Physiology and Kinesiology, University of Florida, Gainesville, Florida

Abstract

Friedreich’s ataxia (FA) is an inherited disease that causes degeneration of the nervous system. Features of FA include proprioceptive and cerebellar deficits leading to impaired muscle coordination and, consequently, dysmetria in force and time of movement. The aim of this study is to characterize dysmetria and its association to disease severity. Also, we examine the neural mechanisms of dysmetria by quantifying the EMG burst area, duration, and time-to-peak of the agonist muscle. Twenty-seven individuals with FA and 13 healthy controls (HCs) performed the modified Functional Ataxia Rating Scale and goal-directed movements with the ankle. Dysmetria was quantified as position and time error during dorsiflexion. FA individuals exhibited greater time but not position error than HCs. Moreover, time error correlated with disease severity and was related to increased agonist EMG burst. Temporal dysmetria is associated to disease severity, likely due to altered activation of the agonist muscle. NEW & NOTEWORTHY For the first time, we quantified spatial and temporal dysmetria and its relation to disease severity in Friedreich’s ataxia (FA). We found that FA individuals exhibit temporal but not spatial dysmetria relative to healthy controls. Temporal dysmetria correlated to disease severity in FA and was predicted from an altered activation of the agonist muscle. Therefore, these results provide novel evidence that FA exhibit temporal but not spatial dysmetria, which is different from previous findings on SCA6.

Funder

Friedreich's Ataxia Research Alliance

Publisher

American Physiological Society

Subject

Physiology,General Neuroscience

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