Illustrations
Figure 1
Sleep EEG changes in Patient 1. (A) At six years and 10 months of age, the most predominant spike focus was in the right occipital region. (B) At seven years and seven months of age, frequent spike-waves appeared diffusely with frontopolar and parieto-occipital predominance. (C) At eight years and two months of age, CSWS was confirmed based on whole-night EEG recording (SWI= 95%). (D) At 10 years and nine months of age, CSWS was resolved and only low-amplitude spikes were shown in the left frontopolar region.
Figure 1
Figure 2
Ictal video-EEG of atypical absence seizures in Patient 1. The video shows the patient eating a meal and then suddenly stopping, remaining in the same posture until the spike-wave discharges disappear.
Figure 2
Figure 3
The relationship between EEG findings and IQ values. The IQ values dropped significantly during the period of CSWS in both patients and increased after the disappearance of CSWS in Patient 1. Although Patient 2 did not undergo an IQ test after the disappearance of CSWS, behaviour and school achievement improved significantly. FSIQ: full scale IQ; VCI: Verbal Comprehension Index; PRI: Perceptual Reasoning Index; F to BT-CS: focal to bilateral tonic-clonic seizures; FS: focal seizures, SWI: spike-wave index.
Figure 3
Figure 4
Sleep EEG changes in Patient 2. (A) At four years and two months of age, the most predominant spike EEG foci were recorded in both parieto-occipital regions. (B) At six years and 0 months of age, frequent multifocal spike-waves appeared predominantly over both frontopolar and parieto-occipital regions, both synchronously and asynchronously. (C) At seven years and 0 months of age, CSWS with left hemispheric predominance was confirmed based on whole-night EEG recording (SWI= 90%). (D) At 12 years and 11 months of age, CSWS resolved and only infrequent low-amplitude sharp-wave discharges remained in the left temporal region.
Figure 4
Figure 5
Fp-O spike-peak latency study. In Patient 1, the EEG data averaged (n =10) at the Fp1 and O1 trigger points demonstrated O1 spikes preceding Fp1 spikes by 30 and 50 mseconds, respectively. In Patient 2, P3 spikes always preceded Fp1 spikes by 30 mseconds and 35 mseconds, respectively. The EEG data were sampled from CSWS in both patients.
Figure 5
Auteurs
1 Department of Pediatrics, Tokyo Women's Medical University, Tokyo
2 Yotsugi Medical Center for the Severely Disabled, Tokyo, Japan
* Correspondence: Hirokazu Oguni
Department of Pediatrics,
Tokyo Women's Medical University,
8-1 Kawada-Cho, Shinjuku-ku,
Tokyo 162-8666, Japan
Aim We report two patients with Panayiotopoulos syndrome (PS) who developed encephalopathy related to status epilepticus during slow sleep (ESES) at the peak of their clinical course.