Background: Muscle jerks and their cortical correlates usually occur abruptly and arrhythmically in patients with positive or negative myoclonus. However, there have been several reports in which oscillatory pre-myoclonic cortical discharges occur in patients with repetitive myoclonus (Mov Disord 6 (1994) 633; Brain 119 (1996) 1307; Brain 124 (2001) 2459; Brain 126 (2003) 326). In the present paper, we describe 4 patients with non-repetitive positive or negative myoclonus whose jerks were preceded by 16-20 Hz oscillatory EEG potentials over the contralateral sensorimotor cortex.
Methods: Jerk-locked averaging (JLA) was performed in 3 patients with positive myoclonus two of which had benign familial myoclonus epilepsy whilst the other had tuberculous meningitis. Silent period locked averaging (SPLA) was performed in a patient with hepatic encephalopathy and asterixis.
Results: In the 3 patients with positive myoclonus, JLA revealed a sequence of 20 Hz EEG potentials preceding the myoclonus. The positive peak of the last oscillation preceded the onset of myoclonus by the cortico-muscular latency. In the patient with hepatic encephalopahty, SPLA showed that 16 Hz EEG oscillations over the contralateral motor cortex were associated with small EMG oscillations, and that the largest EEG wave of the oscillation preceded the onset of a large EMG discharge just prior to the EMG silence. These oscillatory activities are similar in frequency to the motor cortical oscillations seen in monkeys and humans during voluntary contraction.
Conclusions: Abnormally enhanced rhythmic (16-20 Hz) activities in the motor cortex are associated with arrhythmic positive or negative myoclonus. The rhythmic activities may also be responsible for the generation of the tremulousness (or mini-asterixis) in metabolic encephalopahty.