High-frequency component in flash visual evoked potentials in type 3 Gaucher disease.

OBJECTIVE

To characterize the visual evoked potentials (VEP) in patients with type 3 Gaucher disease (GD) with or without progressive myoclonus epilepsy.

METHODS

Three young adults with progressive myoclonus epilepsy (type 3a GD) and two children without progressive myoclonus epilepsy (type 3b GD) were enrolled. Flash visual and somatosensory evoked potentials (F-VEP and SEP, respectively) were retrospectively reviewed in all patients under enzyme replacement therapy. Pattern reversal visual evoked potentials (PR-VEP) were recorded in the type 3a group.

RESULT: High-frequency components were provoked at early latencies on averaged F-VEP in all patients with type 3a and one patient with type 3b GD. Conversely, no activities were recorded in PR-VEP. The onset latency of the components began at 15.3-19.8 ms after flash stimulation. Four-five of the F-VEP examination the activities' duration overrode the N75 waveforms and were prolonged toward the P100 peak latency. The F-VEP amplitude did not differ between the type 3a and type 3b groups, while the SEP amplitude was higher in the type 3a than in the type 3b group.

SIGNIFICANCE: High-frequency components on F-VEP using bandpass filter at 10-200 Hz may help assess augmented excitability in the visual cortex of type 3 GD patients.