Category Ranking
98%
Total Visits
921
Avg Visit Duration
2 minutes
Citations
20
Article Abstract
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. . 2024;20(8):1227-1232.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11294132 | PMC |
| http://dx.doi.org/10.5664/jcsm.11078 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Long-term impact of growth hormone therapy on mortality and type 2 diabetes in Prader-Willi syndrome: a nationwide cohort study.
Front Endocrinol (Lausanne)
September 2025
Department of Pediatrics, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.
Background: Prader-Willi syndrome (PWS) is a rare genetic disorder characterized by severe multisystem comorbidities and increased mortality. Although growth hormone therapy (GHT) is widely used as standard care, population-based evidence on its long-term safety, particularly in relation to mortality and type 2 diabetes mellitus (T2DM), remains limited. We aimed to investigate the associations between GHT duration, mortality, and T2DM incidence in PWS.
View Article and Find Full Text PDFDevelopment of disease-specific growth charts for Argentine Prader-Willi syndrome without growth hormone treatment.
Ann Hum Biol
December 2025
Growth and Development Department, Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina.
Background: Prader-Willi syndrome (PWS) is a complex genetic disorder characterised by infantile hypotonia, early-onset obesity, intellectual disability, hypopigmentation, small hands and feet, short stature, hypogonadism, and distinctive facial features.
Aim: To generate and report growth curves for height, sitting height, hands and feet length for Argentine children with Prader-Willi syndrome (PWS) without growth hormone treatment.
Subjects And Methods: A total of 1174 anthropometric measures were obtained from 167 children (82 boys) aged 0-19 years attending Hospital Garrahan between 1992 and 2019.
Maternal uniparental disomy of chromosome 15 with concurrent paternal non-chromosome 15 marker chromosome: a rare presentation of prader-willi syndrome.
Mol Cytogenet
August 2025
Assisted Reproduction Unit, Department of Obstetrics and Gynecology, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, 310016, Zhejiang, China.
Background: Prader-Willi Syndrome (PWS) is a complicated genetic disorder demonstrating a variety of clinical phenotypes. Using molecular cytogenetics approaches to detect the deletions of the paternal 15q11-q13 region and maternal uniparental disomy of chromosome 15 plays an important role in the prenatal diagnosis of PWS.
Case Presentation: A pregnant woman with advanced maternal age underwent amniocentesis.
Sex Hormone Replacement Therapy and Bleeding Patterns among Adolescents and Young Adult Females with Prader-Willi Syndrome.
J Pediatr Adolesc Gynecol
August 2025
University of Colorado Anschutz Medical Campus, Department of Pediatrics, Section of Endocrinology, Children's Hospital Colorado, Aurora, CO, USA.
Introduction: Prader-Willi Syndrome (PWS) is associated with hypogonadism. Limited data exist on guidelines for sex hormone replacement therapy (HRT) in females with PWS. We aimed to characterize pubertal timing, vaginal bleeding patterns, and HRT practices in adolescent and young adult females with PWS.
View Article and Find Full Text PDF