Multiple saccadic abnormalities in spinocerebellar ataxia type 3 can be linked to a single deficiency in velocity...

The purpose of the current research is to understand if the different eye movement abnormalities in patients with the same neurologic disease are related to varied disease processes or, alternately, do different patients adopt different strategies to overcome a singular brain deficiency.

Using a magnetic search coil, we measured saccade dynamics, that is position and velocity waveforms, for patients diagnosed with spinocerebellar ataxia type 3 (SCA-3), also known as Machado-Joseph disease (MJD).

We observed that the saccadic waveform of the majority of the SCA-3 patients (7 of 10) exhibited dynamic overshoot, with the eye passing the desired endpoint and making a rapid correction before coming to rest. Patients with normal waveforms, that is with no dynamic overshoot, had saccades with relatively low peak velocity.

Velocity feedback in a closed loop control system is essential for providing a fast response without overshoot. Lack of a velocity feedback or an imbalance between position and velocity gains yields a tradeoff between response time and overshoot. While the goal of a saccade is to get to the desired position, models based on animal research suggest that the saccadic control also incorporates a velocity feedback. Results presented here indicated that all SCA-3 patients had deviations in the saccadic waveform, albeit of two types, either slow saccades or dynamic overshooting saccades. Using saccadic models based on animal research can explain how a single deficit, that is a mismatched velocity control of the motor error due to the disease, can yield these two different abnormalities in human patients.