PMC9062211_01
Disease course and EEG findings. Abbreviations: SS: slow-spike-and-wave, SSW: spike-and-slow wave.
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PMC9062211_02
Wake and sleep EEG recordings showing suppression phases in wake and burst suppression pattern during sleep. A) Diurnal polygraphic awake recording showed diffuse spike-and-slow wave or polyspike-and-slow wave discharges; on two occasions, a bilateral brief contraction of proximal and truncal muscles lasting 1-2 s (spasm) was recorded with no related epileptiform activity. B) Prolonged monitoring of sleep-wake cycle in wake showed bursts with frequent paroxysmal activities of diffuse spike-and-slow waves or polyspike-and-slow-waves lasting 10-15 s followed by symmetric voltage attenuation lasting 1 second. C) Prolonged monitoring of sleep-wake cycle during NREM 2 stage showed a burst-suppression pattern with phases of suppression lasting up to 4 s during sleep. Abbreviations: Abd.: abdominal respirogram, Delt: deltoid muscle, ECG: electrocardiogram, Ext. Carpi: extensor carpi muscle; L.: left, Orb.: orbicularis oculi muscle, R., right.
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Several spasms and asynchronous myoclonic jerks were recorded (Fig. 2A-B). EEG recordings were characterized by a gradual increase of suppression phases duration, which morphed into a burst suppression (BS) pattern in NREM stage 2 and REM (Fig. 2C).