Advertisement

Three decades of sociomedical experiences from West Swedish Rett females 4–60 years of age

      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

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Brain and Development
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

      1. Hagberg B. Rett syndrome – clinical and biological aspects. Clinics in developmental medicine. MacKeith Press, Cambridge1993
        • Hagberg B.
        • Berg M.
        • Steffenburg U.
        Rett syndrome, an odd disorder affecting girls: update of 25-year follow-up in Western Sweden.
        Läkartidningen. 1999; 96: 5488-5490
        • Steffenburg U.
        • Hagberg G.
        • Hagberg B.
        Epilepsy in a representative series of Rett syndrome.
        Acta Paediatr. 2001; 90: 34-39
        • Armstrong D.
        • Kay Dunn J.
        • Antalffy B.
        • Trivedi R.
        Selective dendritic alterations in the cortex of Rett syndrome.
        J Neuropathol Exp Neurol. 1995; 54: 195-201
        • Belichenko P.V.
        • Hagberg B.
        • Dahlström A.
        Morphological study of neocortical areas in Rett syndrome.
        Acta Neuropathol. 1997; 93: 50-61