Cortical reactivity in progressive myoclonus epilepsy.

We studied 4 patients with progressive myoclonus epilepsy (Unverricht-Lundborg disease; ULD). Somatosensory evoked fields (SEFs), auditory evoked fields (AEFs), and spontaneous activity over the somatomotor cortex were recorded with a 24-channel SQUID gradiometer. All patients had "giant" 20-45 msec median nerve SEFs at the first somatomotor cortex, with 2-6 times larger amplitudes than the healthy control subjects. Later deflections were not similarly enhanced. The dependence of SEF amplitudes on interstimulus interval (0.2-4 sec) and on successive ulnar-median nerve stimulation (stimulus interval 40 msec) was comparable to that in controls. Cortical AEFs were attenuated and delayed. In 3 patients, the spontaneous activity consisted of 6-8 Hz mu rhythm, which originated within 2 cm from the sources of SEFs and was abolished by clenching of the contralateral fist. Control subjects had major spectral peaks around 10 and 20 Hz. The SEF amplitudes and the strength of the 6-10 Hz mu correlated strongly, suggesting that some components of evoked and spontaneous activity obtain contributions from overlapping neuronal populations. The results imply that ULD is associated with thalamo-cortical hyperreactivity in the sensorimotor but not in the auditory system.