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APNEA, CENTRAL SLEEP

APNEA, CENTRAL SLEEP

Alternative titles; symbolsSLEEP APNEA, LETHAL CENTRALAdickes et al. (1986) described lethal sleep apnea in 4 of 6 sibs, including a pair of twin girls. The firs...

Alternative titles; symbols

  • SLEEP APNEA, LETHAL CENTRAL

Adickes et al. (1986) described lethal sleep apnea in 4 of 6 sibs, including a pair of twin girls. The first twin developed normally until the age of 25 months when, during sleep, she developed irregular respirations, perioral cyanosis, flaccidity, and urinary incontinence. Several other similar episodes followed during one of which the child died at the age of 27 months. The second twin first displayed sleep apnea at age 27 months, shortly after the death of her twin sister. She died after an apneic episode at the age of 31 months. A brother died at 18 months after a single episode of sleep apnea. The fourth affected sib, a girl, was evaluated for apneic tendency at 7 weeks of age, because of the experience with the previous 3 sibs. She was found to be affected. Between 2 and 31 months of age, numerous hospitalizations were required for apneic episodes, some with severe lactic acidosis. Inhibitor substance to thiamine triphosphate (TTP) was present in the urine. The child died at age 31 months as a result of a severe apneic spell. Adickes et al. (1986) concluded that this may be a form of subacute necrotizing encephalomyopathy (Leigh disease; 256000). Lesions were confined to the respiratory centers of the lower brain stem.

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