The sleep and respiratory patterns associated with this disorder have been studied in 11 females aged 2 through 15 years at the Methodist Hospital, Houston, Tx. Polygraphic recordings showed a pattern of disorganised breathing and compensatory hyperpnea during wakefulness with regular, continuous breathing during sleep. The findings suggest an altered or impaired voluntary/behavioural respiratory control system in patients with Rett’s Syndrome. [1]
COMMENT: In 1966, Rett described a progressive dementia in girls with onset in early childhood and associated with autistic behaviour, apraxia of gait, and stereotyped use of the hands. The cause of Rett’s syndrome is unknown. I have seen several atypical cases that fit the description except for the absence of so-called pathognomonic hand wringing movements and hyperventilation, and some were boys. Is Rett’s syndrome a specific disorder or nonspecific, with more than one etiology? For a review of Rett syndrome, refer to Ann J Med Genet 1986 (suppl).