Lack of activation of human secondary somatosensory cortex in Unverricht‐Lundborg type of progressive myoclonus epilepsy

Previous electroencephalographic and magnetoencephalographic studies have demonstrated giant early somatosensory cortical responses in patients with cortical myoclonus. We applied whole‐scalp magnetoencephalography to study activation sequences of the somatosensory cortical network in 7 patients with Unverricht‐Lundborg–type progressive myoclonus epilepsy diagnostically verified by DNA analysis. Responses to electric median nerve stimuli displayed 30‐msec peaks at the contralateral primary somatosensory cortex that were four times stronger in patients than in control subjects. The amplitudes of 20‐msec responses did not significantly differ between the groups. In contrast to control subjects, 5 patients displayed ipsilateral primary somatosensory cortex activity at 48 to 61 msec in response to both left‐ and right‐sided median nerve stimuli. Furthermore, their secondary somatosensory cortex was not significantly activated. These abnormalities indicate altered responsiveness of the entire somatosensory cortical network outside the contralateral primary somatosensory cortex in patients with Unverricht‐Lundborg–type progressive myoclonus epilepsy. The deficient activation of the secondary somatosensory cortex in Unverricht‐Lundborg patients may reflect disturbed sensorimotor integration, probably related to impaired movement coordination. Ann Neurol 2001;49:90–97