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Rett Syndrome

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Abstract

Rett Syndrome is a neurological disorder which was first recognized in 1966 by Professor Andreas Rett as he conducted a routine clinic in Vienna. He noticed two little girls sitting outside his waiting room on their mothers’ laps; both girls were rocking slightly and moving their hands in a strange handwashing motion and their mothers were busy trying to restrain them. This simple observation sparked off Professor Rett’s firm conviction that he had seen other such cases and, put together, the features of all the girls followed the same pattern. Following this observation and much further investigation, Professor Rett’s findings were written up in a German medical publication, which sadly did not get worldwide readership. In the ensuing years Professor Rett toured Europe with a film of the girls, enquiring if such children had been seen by other neurologists. But it was twenty years later that another eminent paediatrician, Professor Bengt Hagberg from Sweden, also recognized this particular group of girls as having strikingly similar symptoms to justify further close examination. This time his efforts, combined with those of associates in Paris and Lisbon, reached a wider audience and (Hagberg et al., 1983) Hagberg’s observations were published in a widely read publication.

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References

  • Hagberg, B., Aicardi, J., Dias, K. and Ramos, O. (1983) A progressive syndrome of autism, dementia, ataxia and loss of purposeful hand use in girls: Rett’s Syndrome, report of 35 cases. Ann. Neurol., 4, 471–9.

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  • Hagberg, B., Goutières. F., Hanefeld, F., Rett, A., and Wilson, J. (1985) Rett Syndrome: criteria for inclusion and exclusion. Brain Dey., 7, 372–3.

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  • Kerr, A.M. (1986) Introduction, comment, observations on 30 cases. The situation for the therapist in Rett Syndrome. Rett Syndrome Glasgow Workshop 1986. J. Mental Deficiency Res.,in press.

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  • Kerr, A.M. and Stephenson, J.B.P. (1985) Rett’s Syndrome in the west of Scotland. Br. Med. J., 291, 579–82.

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  • Rett, A. (1966) Über ein eigenartiges hirnatrophisches Syndrom bei Hyperammonämie im Kindesalter. Wien Med. Wochenschr., 116, 723–6.

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© 1987 Springer Science+Business Media Dordrecht

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Milne, Y. (1987). Rett Syndrome. In: Profound Retardation and Multiple Impairment. Springer, Boston, MA. https://doi.org/10.1007/978-1-4899-7146-3_6

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  • DOI: https://doi.org/10.1007/978-1-4899-7146-3_6

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-0-412-34630-9

  • Online ISBN: 978-1-4899-7146-3

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