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14 and 6 Hz like spike wave activity is a common finding in young patients with Prader-Willi syndrome
Mohammed Alzaid1,2, Kanokkarn Sunkonkit1,3, Colin Massicotte1
1Division of Respiratory Medicine, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
Insights
The 14 and 6 Hz like spike wave activity EEG variant is common in children with Prader-Willi syndrome (PWS). This common EEG finding in PWS patients was not linked to sleep-disordered breathing.
Area of Science:
- Neurophysiology
- Pediatric Neurology
- Sleep Medicine
Background:
- The 14 and 6 Hz like spike wave activity is an electroencephalogram (EEG) variant.
- Its prevalence and clinical significance in pediatric Prader-Willi syndrome (PWS) are not well-established.
- This study investigates this EEG variant in children with PWS.
Purpose of the Study:
- To characterize the 14 and 6 Hz like spike wave activity on EEG in children with Prader-Willi syndrome (PWS).
- To evaluate the association between this EEG variant and sleep-disordered breathing (SDB) indices in children with PWS.
Main Methods:
- Retrospective review of EEGs from children with PWS and healthy controls who underwent polysomnography.
- Analysis of the prevalence, characteristics, and clinical correlation of the 14 and 6 Hz like spike wave activity.
- Comparison of SDB indices between children with and without the EEG variant.
Main Results:
- The 14 and 6 Hz like spike wave activity was present in 51% of children with PWS, versus 0% in healthy controls.
- The EEG variant was bilateral in 52%, predominantly frontal in 48%, and occurred during NREM stage 2 sleep in 52%.
- No correlation was found between the EEG variant and SDB indices in children with PWS.
Conclusions:
- The 14 and 6 Hz like spike wave activity is a frequent EEG finding in pediatric Prader-Willi syndrome.
- This EEG variant is not associated with sleep-disordered breathing in children with PWS.
- The clinical significance of this EEG variant in PWS remains undetermined.
Study Objectives:
Our aim was to characterize the 14 and 6 like spike wave activity seen on electroencephalograms (EEG) in children with Prader-Willi syndrome (PWS) undergoing polysomnograms.
Methods:
We performed a retrospective review of children with PWS and healthy controls who underwent diagnostic polysomnograms between January 1, 2007, and December 31, 2020, at SickKids, Toronto, Canada. EEGs from the polysomnograms were reviewed for the presence of the 14 and 6 like spike wave activity and its characteristics. Clinical correlation of the EEG variant with sleep-disordered breathing indices from the polysomnograms was also evaluated.
Results:
A total of 94 children with PWS and 50 healthy controls were included. The median age and interquartile range for the cohort was 1.42 (0.6, 4.2) years. There were 50 (53.2%) males in the PWS cohort. The EEG variant prevalence in this cohort was 51.0% (n = 48) in children with PWS and 0% for the healthy controls. 14 and 6 Hz like spike wave activity was bilateral in 52% (25/48) children with PWS. The waves had a negative deflection in almost all patients, 44/48 (92%), with PWS. It was predominantly located in the frontal leads for children with PWS, 23/48 (47.9%). It most frequently occurred during non-rapid eye movement stage 2 sleep for children with PWS, 25/48 (52.0%). The mean (standard deviation) frequency was 6.8 (0.97) Hz. The median (interquartile range) length of the waves was 1.1 (0.8, 1.4) seconds in children with PWS. There was no correlation between the presence of the EEG variant and sleep-disordered breathing indices in children with PWS.
Conclusions:
The 14 and 6 Hz like spike wave activity EEG variant was present in more than 50% of a pediatric cohort of children with PWS compared with 0% in healthy children. This EEG variant did not appear to be associated with sleep-disordered breathing indices in children with PWS and is of unknown clinical significance.
Citation:
Alzaid M, Sunkonkit K, Massicotte C, Otsubo H, Amin R, Al-Saleh S. 14 and 6 Hz like spike wave activity is a common finding in young patients with Prader-Willi syndrome. J Clin Sleep Med. 2024;20(8):1227-1232.
