Different neurophysiologic patterns of myoclonus characterize Lennox-Gastaut syndrome and myoclonic astatic epilepsy.

Abstract:

PURPOSE:To study the neurophysiologic characteristics of epileptic myoclonus in patients with Lennox-Gastaut syndrome (LGS) and myoclonic astatic epilepsy (MAE). METHODS:Three patients with symptomatic LGS (mean age, 15 years +/- 4) and three with cryptogenic MAE (mean age, 9 years +/- 3) were studied. Temporal relationships between electroencephalographic (EEG) and electromyographic activity were studied by analyzing latencies of EEG activity related to the onset of single myoclonic jerks, by using burst-locked EEG averaging where necessary. RESULTS:LGS: neurophysiologic analysis indicated that jerks and the accompanying premyoclonic spikes showed latency differences between sides (mean +/- SD, 18 +/- 5 ms for both) with a constant leading side in each patient. The premyoclonic spike latency was 20 +/- 10 ms (mean +/- SD). Topographic voltage mapping of the premyoclonic spike peak showed a unilateral frontal distribution. MAE: muscles from both sides were activated synchronously, and the EEG correlate was a generalized spike-wave, in which the negative peak of the spike preceded the generalized jerks by 30 +/- 2 ms (mean +/- SD). Topographic voltage mapping of the premyoclonic spike peak showed a diffuse distribution of the electrical field, predominating over the anterior regions, but not lateralized. CONCLUSIONS:These neurophysiologic findings indicate that epileptic myoclonus in LGS originates from a stable generator in the frontal cortex, to spread to contralateral and ipsilateral cortical areas, whereas myoclonus in MAE appears to be a primary generalized epileptic phenomenon.

journal_name

Epilepsia

journal_title

Epilepsia

authors

Bonanni P,Parmeggiani L,Guerrini R

doi

10.1046/j.1528-1157.2002.30301.x

subject

Has Abstract

pub_date

2002-06-01 00:00:00

pages

609-15

issue

6

eissn

0013-9580

issn

1528-1167

pii

epi30301

journal_volume

43

pub_type

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