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Immaturity of medullary cardiorespiratory neurones leading to inappropriate autonomic reactions as a likely cause of sudden death in Rett’s syndrome
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Editor,—Rett’s syndrome is a cause of intellectual disability with frequent respiratory dysrhythmia. In a survey in Great Britain, we identified episodic hyperventilation in 75%, apnoeic attacks in 70%, non-seizure vacant spells in 77%, and epileptic seizures in 70% of 191 classic cases. Of all reported deaths from Rett’s syndrome, 25% were sudden and unexpected. Certain groups of interacting neurones in the medulla perform autonomic and respiratory functions that can be measured non-invasively. With informed consent and the approval of the local ethics committee, we measured the autonomic reactions …