Neurophysiology of myoclonus

Abstract

Myoclonus may be generated by any area in the central nervous system. Finding its generator is helpful in the diagnostic process. Although clinical features have to be carefully analyzed as they may give a first idea, neurophysiologic study of myoclonus provides the most important clues for the determination of the generator. Surface electromyography (EMG) allows analyzing the recruitment order in generalized myoclonus, thereby suggesting either a cortical, brainstem, or spinal origin. It also reveals whether myoclonus is positive (jerks that are caused by muscle activation) or negative (jerks that are caused by brief muscle inhibition). In non-generalized myoclonus the EMG burst duration gives an idea of the level of the generator. Repetitive peripheral nerve stimulation is required to record somatosensory evoked potentials (SEPs) as well as long latency reflexes (LLR), especially the C reflex. The presence of giant cortical SEPs is an indirect argument for cortical myoclonus. Similarly the existence of LLR at rest orientates towards cortical reflex (sensitive to sensory stimuli) myoclonus. Finally EEG-EMG polygraphy is the only test which is able to prove directly the cortical origin of myoclonus. This is the case when focal cortical events precede myoclonus with a fixed delay. These premyoclonic cortical potentials may either be seen directly on raw recordings or require the use of jerk-locked back averaging (JLBA). This technique allows the averaging of the EEG prior to myoclonus onset (as determined by EMG) in order to reveal a premyoclonic spike that otherwise would remain undetected in the global EEG.