Rett syndrome and ageing: a case study.
OBJECTIVE
This case study of an elderly women with Rett syndrome is used to consider whether observed changes may be related to physiological processes involved in ageing or environmental adaptations, which is important for delivery of rehabilitation.


METHODS
The life story of a woman with Rett syndrome who lived to the age of 60 is presented. It is based on medical records, older and more recent videotapes, and interviews with her sister and care staff.


RESULTS
After 21 years without walking, following intensive physiotherapy, she regained the ability to walk without support. She also showed improvement in hand use a few years before she died. During the early regression she appeared to lose social interest. The interest improved after some time, but she remained wary of people she did not know.


CONCLUSIONS
The walking and hand use indicate that these functions may have been present to a greater extent than assumed by people in the environment and that her poor function reflects dyspraxia and lack of opportunity and training rather than lack of ability. Although more studies of elderly women with Rett syndrome is needed to answer whether the observed changes were due to physiological processes involved in ageing or environmental adaptations, the present case story demonstrates that identification of females with Rett syndrome is important at all age levels.
Department of Psychology, University of Trondheim (NTNU), Norway. Karl.Jacobsen@svt.ntnu.no
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Jacobsen
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Viken
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von Tetzchner
D000203:Activities of Daily Living; D000223:Adaptation, Psychological; D000375:Aging / Q000502:physiology / Q000523:psychology; D005260:Female; D006225:Hand / Q000503:physiopathology; D006801:Humans; D008875:Middle Aged; D012189:Retrospective Studies; D015518:Rett Syndrome / Q000150:complications / Q000503:physiopathology / Q000523:psychology / Q000534:rehabilitation*; D012648:Self Care; D012919:Social Behavior; D011795:Surveys and Questionnaires; D014743:Videotape Recording; D016138:Walking