2013年2月16日 星期六

Thalamocortical myoclonus

The following sections about thalamocortical myoclonus was excerpted from Eplilesy textbook on 2013/02/16
Thalamocortical myoclonus or idiopathic generalized epileptic myoclonus represents the common type of myoclonus in various epileptic syndromes. Myoclonia are often spontaneous, predominantly arrhythmic and axial with varying severity, and associated chronologically with an EEG pattern of diffuse polyspikes or (poly) spike-and-wave discharges. A hyperexcitable cortex is thought to be driven diffusely and synchronously by ascending subcortical inputs that trigger the paroxysmal events. As a consequence, muscles from both sides are activated, and muscles innervated by the cranial nerves are involved through a rostrocaudal manner.
Electrophysiologically, SEP usually does not show giant SEP, and C-reflex may be recorded at rest. A negative peak of the generalized spike (30–100 msec duration) precedes the jerk (<100 msec duration) by 20 to 75 msec. The latency of the spike is relatively longer, and the temporal relationship is looser than in that of cortical myoclonus. The underlying mechanism of the thalamocortical myoclonus is still uncertain. The myoclonus of benign myoclonic epilepsy of infancy, myoclonic-astatic epilepsy, and JME belongs to this category.
Myoclonus observed in patients with Dravet syndrome is not straightforward: Patients may exhibit massive myoclonus combined with a generalized spike-wave (rarely in infancy, mostly in childhood), and erratic myoclonus, particularly during episodes of myoclonic status, in which the patient is drowsy with diffuse slow wave activity and few spikes. The generator remains unidentified.

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