A similar situation happens in patients with Ondine's curse, … Yasuma F, Nomura H, Sotobata I, Ishihara H, Saito H, Yasuura K, Okamoto H, Hirose S, Abe T, Seki A. Congenital central alveolar hypoventilation (C-CAH), so called Ondine's curse, is known to be quite a rare neuropathology that has been reported in only 23 cases to date. There are only a few cases-about 200 to 500 worldwide-each year. Working off-campus? The other conditions frequently associated with Ondine's curse were: brainstem disturbances, Hirschsprung disease and…, Congenital central hypoventilation syndrome (Ondine's curse syndrome) in two siblings: Delayed diagnosis and successful noninvasive treatment, Medullary Infarction with Central Hypoventilation ( Ondine ’ s Curse ) : A Case Report, Congenital central hypoventilation syndrome associated with Hirschsprung's disease and neuroblastoma: Case of multiple neurocristopathies. A 9‐month‐old child was struck with television directly on the head. Neither did my patient with Ondine’s Curse have mountain sickness. He is currently a 7‐year‐old child who is ventilator dependent at home at night, but during the daytime, breathes well. The term "Ondine's curse" is more commonly used for cases of congenital central alveolar hypoventilation, but it can also be applied to acquired cases 8. It is always congenital, meaning that it is present from birth. Semantic Scholar is a free, AI-powered research tool for scientific literature, based at the Allen Institute for AI. The nymph Ondine was an immortal water spirit who became human after falling in love for a man, marrying him, and having a baby. Ondine's curse associated with Hirschsprung disease and ganglioneuroblastoma. Ondine's curse was mentioned in the 1987 film Black Widow starring Debra Winger, Theresa Russell, Terry O'Quinn and D. W. Moffett. Cases of Ondine's curse and Hirschsprung's disease. Ondine's curse, also called congenital central alveolar hypoventilation (CCAH), is a rare condition involving failed automatic respiration in the absence of cardiopulmonary disease, resulting in inadequate ventilation with progressive hypercapnia and hypoxia during sleep . Congenital Central Hypoventilation Syndrome (CCHS), also referred with the expression "Ondine's Curse", is a rare genetic life-long disease resulting from the mutation of PHOX2B gene on chromosome 4p12.3. infarction complicated Ondine's curse. Terao reported a case of right medullary dorsolateral and bilater - al cerebellar infarction with Ondine’s curse and rapid respiratory failure [10]. 1) . Enter your email address below and we will send you your username, If the address matches an existing account you will receive an email with instructions to retrieve your username. Materials and methods: This is a report of 3 cases of LMS with Ondine's curse. In 1990, Bogosslavsky et al reported two cases of respiratory failure caused by dorsolateral infarction of the medulla oblongata [9]. Ondine’s Curse after High Cervical Cordotomy. Ondine's curse is a rare and potentially fatal disorder characterised by loss of automatic alveolar hypoventilation during non‐rapid eye movement (REM) sleep and improves during REM sleep and quiet wakefulness.1 Affected patients develop hypoxaemia and hypercarbia, but lack a stimulation response to either. Typically the disorder is congenital, but in some cases can be developed later in life, especially when the patient has also had a severe injury to the brain or brain stem. Congenital central hypoventilation syndrome and Hirschsprung’s disease, Care of the Child with Congenital Central Hypoventilation Syndrome. The investigations and treatment for these syndromes are discussed. Sleep apnea is a potentially fatal complication after percutaneous cervical cordotomy. Central hypoventilation syndrome (“Ondine’s Curse”) is an infrequent disorder that can lead to serious acute or chronic health consequences. Please check your email for instructions on resetting your password. Case report A 67-year-old female was admitted to our hospital for diz-ziness, slurred speech and unsteady walk. In majority of the cases, Ondine’s Curse is present at the time of the birth of the child, but in some cases it may develop after an injury to the brain where the brainstem gets injured. He subsequently deteriorated neurologically with radiographic evidence of progressive triventriculomegaly. Therefore, he was condemned to remain awake forever. All had diabetes mellitus We describe the case of recurrent sleep apnoea associated with pregnancy and delivery and related to a Chiari malformation that became symptomatic only during pregnancy. Typically the disorder is congenital, but in some cases can be developed later in life, especially when the patient has also had a severe injury to the brain or brain stem. Ondine's curse was also mentioned in the book It's Kind of A Funny Story (Ned Vizzini). Ondine's Curse was given as a reason for the death of a mafia connected man who suddenly died in his sleep for no reason. Ondine's curse is one of the most enchanting mythical tales in the field of Medicine. Results: All patients were male and they were 63, 65 and 84 y old respectively. All were on mechanical ventilation. The nymph Ondine was an immortal water spirit who became human after falling in love for a man, marrying him, and having a baby. Treatment includes a tracheotomy and ventilator support. I have read and accept the Wiley Online Library Terms and Conditions of Use, Ondine's curse‐failure of respiratory center automaticity while awake, An official ATS clinical policy statement: congenital central hypoventilation syndrome: genetic basis, diagnosis, and management, Ondine's curse after brainstem infarction, Television‐related injuries to children in the United States, 1990–2011. He underwent tracheostomy on Day 20 of hospital stay. He was placed on acetazolamide for central hypoventilation. An emergency ventriculostomy tube was placed. Ondine’s curse; General Discussion. ... Acquired Ondine's curse: case report. The condition can be fatal if untreated. PMID: 11218702 However, cases of acquired Ondine™s curse have been reported. Cardiovascular Abnormalities and Arrhythmias in Patients with Ondine's Curse (Congenital Central Hypoventilation) Syndrome. Overnight polysomnography revealed prolonged central apnoeas as soon as the patient fell asleep. During the … er, unilateral medullary infarction also led to Ondine’s curse. Ondine’s curse—more appropriately known as congenital central hypoventilation syndrome or CCHS—is a rare, severe form of sleep apneain which an individual arterial blood gas, when the patient was on pressure support ventilation, showed persistent hypercapnia and hypoxia during sleep (PCO2 between 80 and 90 and PO2 under 65) and the gases were normal when he was awake. Materials and methods: This is a report of 3 cases of LMS with Ondine's curse. Congenital central hypoventilation syndrome (CCHS), formerly known as Ondine's curse, is a congenital and severe form of central sleep apnea resulting from abnormal autonomic control of breathing with decreased sensitivity to hypercapnia and hypoxemia, particularly during sleep. The nymph Ondine was an immortal water spirit who became human after falling in love for a man, marrying him, and having a baby. You are currently offline. Use the link below to share a full-text version of this article with your friends and colleagues. Ondine's or Undine's curse is a very rare medical condition characterized by respiratory arrest during sleep. Conflict of interest: The author reports no conflicts of interest. This condition results in loss of automatic and/or voluntary respiration with characteristic polysomnographic finding of impaired ventilator responses to hypercapnia and sleep apnea, which are more pronounced during non-REM sleep, less … Ondine’s curse: a case of anti-Hu paraneoplastic syndrome 3C00 C Brookes, R McIntosh, S Twigg A 60-year-old patient presented with neurological symptoms which proved to be a paraneoplastic syndrome secondary to a small-cell lung cancer. Ondine's curse is associated with respiratory arrests during sleep and, with incomplete penetrance, neuroblastoma (tumors of the sympathetic ganglia), Hirschprung disease (partial agenesis of the enteric nervous system), dysphagia (difficulty swallowing), anomalies of the pupilla, etc. We hypothesise that the generalised oedema that occurred during pregnancy, with a potentially mild increase in intracranial … Ondine’s curse (central hypoventilation syndrome, CHS) is a congenital respiratory disorder developed by birth in patients. Ondine's curse is an eponym that refers to central alveolar apnea/hypopnea observed among patients with acquired or congenital brainstem disorders. 13,14,17,30,41 Sleep apnea has not been associated with low cervical cordotomies. Congenital central hypoventilation syndrome (CCHS) is a rare lifelong and life-threatening disorder. The investigations and treatment for these syndromes are discussed. Ondine’s curse, also known as Central Alveolar Hypoventilation (CAH) syndrome is a rare clinical condition characterized by the loss of automatic respiratory drive, mostly during sleep but also during wakefulness. Learn more. Two types have been described: congenital and acquired CAH. … Ondine curse is one type of sleep apnea syndrome, defined as the selective disturbance of autonomous breathing. Characteristics and clinical presentations of three patients were described and analysed. Peaks of oxygen desaturation reached a nadir of 55%. C-CAH was diagnosed in a 2-year-5-month-old infant. This hypoventilation induced chronic complications, such as a pulmonary arterial hypertension and psychomotor and/or growth retardation. trigger Ondine’s curse in patients with stroke: 1) infarctions which affected automatic respiration system, 2) apnea, mostly during sleep and 3) other suspected risk factors. Ondine’s curse — or, more officially, central hypoventilation syndrome — is an often fatal respiratory disorder that occurs during sleep. The apnoea/hypopnoea index was 107 events/h. Case Report: Ondine's Curse Syndrome Secondary Brainstem Infarction Canmin Zhu, Ming Li *, Jiaping Xiao, Xuan Zhang , Mei Zhang, Qiang Li and Wei Zeng Department of Neurology, The Fifth Hospital of Wuhan, 122 Xianzheng Street, Hanyang, Wuhan, Hu-Bei, P.R. 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). Ventriculostomy tube was removed on Day 10. Some features of the site may not work correctly. Ondine’s curse — or, more officially, central hypoventilation syndrome — is an often fatal respiratory disorder that occurs during sleep. Ondine's curse was mentioned in the 1987 film Black Widow starring Debra Winger, Theresa Russell, Terry O'Quinn and D. W. Moffett. 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