A patient with Rett syndrome may die suddenly. Death determination requires consideration of a number of possible causes and a careful post-mortem exam.
Most patients with Rett syndrome are infants or children.
Possible causes of sudden death in a patient with Rett syndrome:
(1) severe seizures
(2) autonomic dysfunction, causing electrical instability in the heart
(3) prolonged QTc and/or T-wave abnormalities
(4) central hypoventilation or respiratory insufficiency (associated with immaturity of medullary cardiorespiratory neurons)
(5) infection
The drugs that the patient was taking should be reviewed since these could contribute to respiratory depression and/or further prolongation of the QTc.
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