Rett syndrome: analysis of deaths in the British survey

Eur Child Adolesc Psychiatry. 1997;6 Suppl 1:71-4.


The mortality rate in Rett syndrome is 1.2% per annum, 48% of deaths occurring in debilitated people, 13% from natural causes, 13% with prior severe seizures and 26% sudden and unexpected. Respiratory dysrhythmias were usually present. Neuropathology confirmed reductions in cortical dendrites and in one case immaturity of cardiac conducting tissues.

MeSH terms

  • Cause of Death
  • Female
  • Humans
  • Population Surveillance*
  • Prospective Studies
  • Respiration Disorders / mortality
  • Rett Syndrome / mortality*
  • Rett Syndrome / pathology
  • Severity of Illness Index
  • United Kingdom / epidemiology