Biomechanical analysis of simple jaw movements in Friedreich's ataxia

The jaw movement kinematics in relation to the EMG activity of two antagonistic jaw muscles (the masseter and the digastricus pars anterior) were examined in healthy subjects and patients with Friedreich's disease. Dysarthria and ataxia are the main characteristics of this disease. Jaw movement was monitored with a magnetometer system, and bipolar surface electrodes were used to record EMG activity. Unidirectional opening, unidirectional closing and opening-closing movements of the mandible were performed in a simple reaction time situation. The data were compared with those for normal control subjects. The kinematic and electromyographic characteristics were: (a) prolonged total movement duration resulting from increased acceleration and deceleration durations; (b) decreased maximum velocity, and a secondary peak in the velocity profile; (c) tonic EMG activity in muscles supposedly at rest; (d) prolonged EMG bursts. Premotor reaction time was also increased. These characteristics are similar to those, previously described, of limb movements in subjects with cerebellar dysfunctions and suggest that the alterations of jaw movement in Friedreich's ataxia could be due to a deficit in cerebellar control.