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Incidence of classic forms: 1:12000 - 1: 15000
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Incidence of atypical forms: 1: 45000
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In Australia, Rett Syndrome affects about 1 in 15,000 girls aged 5 to 18 years. Approx 12 girls per year, are diagnosed by the age of 12 years and10% of those with RS reside in South Australia. By 2002, there were about 250 girls throughout Australia who had been diagnosed with this condition.
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RS is most often misdiagnosed as autism, cerebral palsy or non-specific developmental delay.
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RS occurs in all races and ethnic groups.
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About 70% survive to age 35 years. The majority of deaths in RS are either sudden and unexpected, or secondary to pneumonia.