Ondine's curse, primary alveolar hypoventilation, alveolar hypoventilation secondary to neurologic disease, idiopathic acquired central hypoventilation syndrome
Ondine by John William Waterhouse (1849–1917)
Specialty
Neurology
Central hypoventilation syndrome (CHS) is a sleep-related breathing disorder that causes ineffective breathing, apnea, or respiratory arrest during sleep (and during wakefulness in severe cases). CHS can either be congenital (CCHS) or acquired (ACHS) later in life. The condition can be fatal if untreated. CCHS was once known as Ondine's curse.
ACHS can develop as a result of severe injury or trauma to the brain or brainstem.[1] Congenital cases are very rare and involve a failure of autonomic control of breathing. In 2006, there were only about 200 known cases worldwide. As of 2008, only 1000 total cases were known.[2] The diagnosis may be delayed because of variations in the severity of the manifestations or lack of awareness in the medical community, particularly in milder cases.[3] However, as there have been cases where asymptomatic family members also were found to have CCHS, it may be that these figures only reflect those found to require mechanical ventilation. In all cases, episodes of apnea occur in sleep, but in a few patients, at the most severe end of the spectrum, apnea also occurs while awake.
Although rare, cases of long-term untreated CCHS have been reported and are termed late onset CCHS (LO-CCHS).[4] There have, however, even been cases of LO-CCHS where family members found to have it have been asymptomatic.[5] Again, lack of awareness in the medical community may cause such a delay.[6] CCHS susceptibility is not known to be affected by sex or race.[3]
^Jazeela Fayyaz, DO (2017-12-05). Zab Mosenifar, M (ed.). "Hypoventilation Syndromes". Medscape.
^"Congenital central hypoventilation syndrome". Genetics Home Reference. U.S. National Library of Medicine. Retrieved 5 June 2015.
^ abCongenital Central Hypoventilation Syndrome at eMedicine
^Windisch W, Hennings E, Storre J, Matthys H, Sorichter S (2004). "Long-term survival of a patient with congenital central hypoventilation syndrome despite the lack of continuous ventilatory support". Respiration. 71 (2): 195–8. doi:10.1159/000076685. PMID 15031579. S2CID 36554567.
^Bygarski, Elizabeth; Paterson, Melanie; Lemire, Edmond G. (April 26, 2013). "Extreme intra-familial variability of congenital central hypoventilation syndrome: a case series". Journal of Medical Case Reports. 7 (1): 117. doi:10.1186/1752-1947-7-117. PMC 3651317. PMID 23622117.
^Lovell BL, Bullock RE, Anderson KN (March 2010). "An unusual presentation of congenital central hypoventilation syndrome (Ondine's Curse)". Emerg Med J. 27 (3): 237–8. doi:10.1136/emj.2009.072215. PMID 20304901. S2CID 206938512.
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