Abstract
Rett syndrome, today known as a worldwide important cause behind severe mental retardation
in females, has been seen in Sweden since the 1960s (the senior author). This study
gives population representative clinical and sociomedical follow-up data from West
Sweden (1/4 of the population in Sweden). The series comprises 54 females diagnosed
in 1971–1998, 5–60 years old, median age 20. Mortality in 1971–2000 was 18% (10/54),
median age at death 20. Death usually had been sudden and unexpected. The sociomedical
follow-up emphasized the adult group. The large majority had usually moved to small
group homes, a good and well-accepted environment once the staff had learned the particularities
of these so uniquely neurodevelopmentally disabled women.
Keywords
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References
- Hagberg B. Rett syndrome – clinical and biological aspects. Clinics in developmental medicine. MacKeith Press, Cambridge1993
- Rett syndrome, an odd disorder affecting girls: update of 25-year follow-up in Western Sweden.Läkartidningen. 1999; 96: 5488-5490
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© 2001 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
