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Epileptic Disorders

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Intractable apnoeic seizures in a child with a deletion typically associated with Williams syndrome Volume 20, issue 6, December 2018

At 13 months of age, video-EEG monitoringwas performed for five days using 19 scalp electrodes with Oz reference, EMG over the bilateral deltoid muscles, a respiratory monitor, ECG, O2 saturation, and pulse rate monitor. Three habitual seizures were captured on the fifth day of video-EEG monitoring. The first seizure occurred at 9:42 pm during REM sleep, the second seizure at 03:43 am during wakefulness, and the third seizure at 5:43 am during non-REM sleep. The duration of these seizures ranged from approximately 1 minute to 1.5 minutes, although the offset of ictal EEG change was unclear due to movement/muscle artefacts. Ictal EEG onset showed a mixture of 15-Hz beta waves and 5-8-Hz spikes over the right frontotemporal region at Fp2-F4 and F8-T4, followed by rhythmic 3-3.5-Hz spike and waves over the right fronto-temporal region at Fp2-F4, F8-T4, and T6. These activities spread to the right hemisphere as 2-3-Hz delta waves, followed by a build-up of rhythmic 4-5-Hz theta waves over the bilateral temporal regions. Clinically, the patient presented with apnoea first, whichwas detected by the respiratory monitor. This was followed by gradual decline of O2 saturation (down to 21% in the first seizure, 49% in the second seizure, and 52% in the third seizure) and gradual increase in heart rate (up to 144 beats per minute in the first seizure, 166 in the second seizure, and 157 in the third seizure). Towards the end of these seizures, the patient started coughing. During the apnoea, he was moving randomly and did not cry. In the first seizure, at 21:42:42, a mixture of lowamplitude 15-Hz beta waves and 5-8-Hz spikes started over the right fronto-temporal region at Fp2- F4 and F8-T4. O2 saturation was 99%, and heart rate was 104 beats per minute (bpm). At 21:42:55, rhythmic 3-3.5-Hz spike-and-waveswere seen over the right fronto-temporal region at Fp2- F4, F8-T4, T6 and C4. O2 saturation was 95%. Heart rate was 97 bpm. At 21:42:57, the respiratory monitor detected low amplitude. At 21:43:00, the patient was aroused from REM sleep and started moving randomly. At 21:43:07, rhythmic 2-3-Hz delta waves spread to the right hemisphere.O2 saturation was 93%. Heart rate was 102 bpm. At 21:43:12, the respiratory monitor showed a flat line. At 21:43:16, rhythmic 4-Hz theta waves were seen over the right hemisphere. O2 saturation was 87%. Heart rate was 126 bpm. At 21:43:23, rhythmic 4-5-Hz theta waves gradually increased with amplitude over the bilateral temporal regions. O2 saturation was 84%. Heart rate was 129 bpm. At 21:43:30, the monitor alarm for O2 saturation sounded.O2 saturation was 80%. Heart rate was 131 bpm. At 21:43:52, a mixture of 2-Hz delta waves and 4-5- Hz theta waves spread diffusely. O2 saturation was 35%. Heart rate was 136 bpm. At 21:43:53, the pushbutton was activated by his mother. At 21:43:57, O2 saturation went down to 21%. Heart rate was 138 bpm. At 21:44:05, the patient started coughing. At 21:44:20, O2 saturation gradually recovered to 58%.

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