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Rett syndrome: geographic variation in prevalence in Norway
O H Skjeldal1, S von Tetzchner, F Aspelund
1Department of Pediatrics, University of Oslo, Rikshospitalet, Norway. olahs@rh.uio.no
Brain & Development
|June 1, 1997
Summary
The prevalence of Rett syndrome in Norway was 2.17 per 10,000 girls, with a higher rate in Rogaland possibly due to genetic clustering. This study included all Rett syndrome variants, ensuring comprehensive case identification.
Area of Science:
- Neurology
- Genetics
- Epidemiology
Background:
- Rett syndrome is a rare neurodevelopmental disorder affecting primarily females.
- Accurate prevalence data is crucial for understanding disease burden and planning healthcare resources.
- Previous prevalence studies may have underestimated the rate due to variations in diagnostic criteria.
Purpose of the Study:
- To determine the prevalence of Rett syndrome in three Norwegian counties.
- To investigate potential geographical variations and underlying causes for observed prevalence rates.
Main Methods:
- A population-based study was conducted in Rogaland, Ostfold, and Nordland counties, Norway.
- Identified all females aged 3-19 years diagnosed with Rett syndrome within the study period.
- Calculated prevalence rates per 10,000 girls based on identified cases and county population data.
Main Results:
- A total of 21 females with Rett syndrome were identified among 96,920 girls aged 3-19.
- The overall prevalence rate was 2.17 per 10,000 girls.
- Rogaland county showed a significantly higher prevalence (3.77 per 10,000 girls) compared to Ostfold (1.05) and Nordland (0.77), suggesting genetic clustering.
Conclusions:
- The study identified a notable prevalence of Rett syndrome in the studied Norwegian counties.
- The higher prevalence in Rogaland is likely attributable to genetic clustering, supported by limited geographical mobility and familial relationships.
- Inclusion of Rett syndrome variants may contribute to the observed prevalence rates.