Ondine's curse was mentioned in the 1987 film Black Widow starring Debra Winger, Theresa Russell, Terry O'Quinn and D. W. Moffett. Ondine’s curse—more appropriately known as congenital central hypoventilation syndrome, or CCHS—is a rare, severe form of sleep apnea in which an individual completely stops breathing when falling asleep. Ondine’s curse—more appropriately known as congenital central hypoventilation syndrome or CCHS—is a rare, severe form of sleep apneain which an individual It is always congenital, meaning that it is present from birth. 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 is one of the most enchanting mythical tales in the field of Medicine. C-CAH was diagnosed in a 2-year-5-month-old infant. Ondine’s Curse after High Cervical Cordotomy. It represents an enormous difficulty in defining a clinical situation where two conditions are represented in different ways, although the underlying mechanism is similar if not identical. Central hypoventilation syndrome (“Ondine’s Curse”) is an infrequent disorder that can lead to serious acute or chronic health consequences. If you do not receive an email within 10 minutes, your email address may not be registered, A 60-year-old patient presented with neurological symptoms which proved to be a paraneoplastic syndrome secondary to a small-cell lung cancer. All were on mechanical ventilation. The full text of this article hosted at iucr.org is unavailable due to technical difficulties. In the treatment of C-CAH, we implanted a right unilateral diaphragm pacemaker in the infant and his respiratory status was remarkably improved after diaphragm pacing. Working off-campus? There have been cases where various members of the same family have … Please check your email for instructions on resetting your password. Ondine's curse is one of the most enchanting mythical tales in the field of Medicine. Congenital central hypoventilation syndrome in children. 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. Neurology India | Mar-Apr 2009 | Vol 57 | Issue 2 207 Pedroso, et al. 13,14,17,30,41 Sleep apnea has not been associated with low cervical cordotomies. 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). Journal of pediatric gastroenterology and nutrition, American journal of respiratory and critical care medicine, Pacing and clinical electrophysiology : PACE, By clicking accept or continuing to use the site, you agree to the terms outlined in our. Computed tomography (CT) head showing subarachnoid haemorrhage in basilar cisterns. Vasospasm, a complication associated with SAH, results in secondary cerebral infarcts. 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. Journal of Pediatric Surgery, 01 Oct 1995, 30(10): 1531 DOI: 10.1016/0022-3468(95)90434-4 PMID: 8786515 . He is currently a 7‐year‐old child who is ventilator dependent at home at night, but during the daytime, breathes well. The name of Ondine's curse is derived from an ancient legend in Germany, where goddess Ondine’s punishment is said to befall those people who You are currently offline. 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. 2020 Apr;78(4):238-240. doi: 10.1590/0004-282X20190162. The rate of paediatric injuries caused by falling TVs is increasing, which emphasises the need for increased preventive strategies.4. Terao reported a case of right medullary dorsolateral and bilater - al cerebellar infarction with Ondine’s curse and rapid respiratory failure [10]. It is usually reported after bilateral high cervical cordotomies, with an incidence of 3 to 5%. In addition, we are not aware of any previously reported cases with associated cranial nerve neuralgias. Case reports describe the acquired conditions in association with damage to the lateral medulla caused by stroke, tumors, and trauma. He had a brief period of cardiorespiratory arrest and was resuscitated and intubated by emergency medical services on scene. CCHS was once known as Ondine's curse. The congenital CAH occurs at birth and is extremely rare China The investigations and treatment for these syndromes are discussed. Barber MD, Scobie WG. He subsequently deteriorated neurologically with radiographic evidence of progressive triventriculomegaly. 1 Affected patients develop hypoxaemia and hypercarbia, but lack a stimulation response to either. It suggested bilateral acute to subacute infarcts in the posterior inferior cerebellar artery (PICA) distributions. … Results: All patients were male and they were 63, 65 and 84 y old respectively. Ventriculostomy tube was removed on Day 10. 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. Learn about our remote access options, Department of Pediatrics, Section of Hospital Pediatrics, West Virginia University Children's Hospital, Morgantown, West Virginia, United States. There was associated T2 signal abnormality in these locations. Materials and methods: This is a report of 3 cases of LMS with Ondine's curse. Ondine's curse: the origin of the myth Arq Neuropsiquiatr. The prognosis of this syndrome is poor and its cause is not fully known. The nymph Ondine was an immortal water spirit who became human after falling in love for a man, marrying him, and having a baby. 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. This hypoventilation induced chronic complications, such as a pulmonary arterial hypertension and psychomotor and/or growth retardation. Of the four, only central alveolar hypoventilation without muscular paralysis is labeled ‘Ondine's curse.’ They state: 'In many cases, a history of encephalitis, trauma, Leigh's disease, Shy-Drager disease, or shock therapy can be obtained from patients with this condition. Characteristics and clinical presentations of three patients were described and analysed. Ondine's curse is a rare form of central respiratory failure which includes severe sleep apnoea (1). We hypothesise that the generalised oedema that occurred during pregnancy, with a potentially mild increase in intracranial … The patient improved 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. Of the four, only central alveolar hypoventilation without muscular paralysis is labeled ‘Ondine's curse.’ They state: 'In many cases, a history of encephalitis, trauma, Leigh's disease, Shy-Drager disease, or shock therapy can be obtained from patients with this condition. Five cases of central congenital hypoventilation, Ondine's curse, were studied. Cardiovascular Abnormalities and Arrhythmias in Patients with Ondine's Curse (Congenital Central Hypoventilation) Syndrome. During his stay in paediatric intensive care unit, there were concerns that he was not initiating breathing over the ventilator. In one of the versions of the tale, when she caught her husband sleeping with another woman, she cursed him to remain awake in order to control his own breathing. 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) is a rare lifelong and life-threatening disorder. Neither did my patient with Ondine’s Curse have mountain sickness. The nymph Ondine was an immortal water spirit who became human after falling in love for a man, marrying him, and having a baby. It arises from Germanic mythology, in which Ondine, an oceanic nymph of incomparable beauty punishes her unfaithful lover with the loss of all movements and functions without not involving his conscious will. Surgical operation and stroke are the reported causes of this syndrome. Therefore, he was condemned to remain awake forever. The diagnostic criteria were: lack of respiratory autonomy during sleep, abnormal CO2 test results and abnormal respiratory monitoring results during sleep. Characteristics and clinical presentations of three patients were described and analysed. 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. 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. : Acquired Ondine’s curse descending medullocervical pathways that subserve automatic breathing can result in Ondine™s curse. There are only a few cases-about 200 to 500 worldwide-each year. The term “Ondine’s curse”is more commonly used for cas-es of congenital central alveolar hypoventilation,but it can also be applied to acquired cases 8.It arises from Germanic mythol-ogy, in which Ondine, an oceanic nymph of incomparable beauty punishes her … The condition can be fatal if untreated. However, cases of acquired Ondine™s curse have been reported. This syndrome, especially in adults, is rare, and even less frequent in the absence of clear pathogenic lesions on MRI. 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. An emergency ventriculostomy tube was placed. Arq … Ondine’s curse has long been recognized as a congenital disorder and can also develop later in life as a result of damage to the lower lateral medulla. During the … [2-4] Lesions selectively involving the ventrolateral . 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. The investigations and treatment for … 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. All had diabetes mellitus Ondine's curse was also mentioned in the book It's Kind of A Funny Story (Ned Vizzini). Brainstem tumor is relatively common cause for children's Ondine curse. The patient improved PMID: 11218702 An emergent computed tomography head was done, which showed a small area of subarachnoid haemorrhage (SAH) (Fig. 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. He was placed on acetazolamide for central hypoventilation. Ondine's curse was mentioned in the 1987 film Black Widow starring Debra Winger, Theresa Russell, Terry O'Quinn and D. W. Moffett. Ondine’s curse — or, more officially, central hypoventilation syndrome — is an often fatal respiratory disorder that occurs during sleep. Some features of the site may not work correctly. 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. Learn more. A 9‐month‐old child was struck with television directly on the head. 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. 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. 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. Summary. 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. Ondine's curse occurred on the 5th, 10th and 23rd d after their strokes. 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. Treatment includes a tracheotomy and ventilator support. Congenital central hypoventilation syndrome and Hirschsprung’s disease, Care of the Child with Congenital Central Hypoventilation Syndrome. Ondine's curse was also mentioned in the book It's Kind of A Funny Story (Ned Vizzini). Conclusion: the Ondine's curse is one of the posterior stroke's presentation characterized by loss of automatic breathing and for the unpredictability of clinical evolution and prognosis. However, it can be acquired later in life if there is considerable damage in the lower lateral medulla of the brain. Magnetic resonance imaging brain demonstrated small foci of diffusion restriction involving the dorsolateral aspect of medulla bilaterally and anteroinferior aspect of both cerebellar hemispheres. Sleep apnea is a potentially fatal complication after percutaneous cervical cordotomy. It is commonly described as congenital hypoventilation syndrome caused by mutations in homeobox gene.2 However, acquired Ondine's curse is seen with lesions in the ventrolateral medulla (infarction, haemorrhage, encephalitis, tumours, mitochondrial disease, demyelination).3 In our case, infarct was likely secondary to vasospasm of the PICAs. 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. In some cases dysphagia, neuroblastoma, and intestinal problems are associated with Ondine’s Curse or Central Hypoventilation Syndrome. CCHS represents an autonomic nervous system disorder; its more fearsome manifestation is centra … Use the link below to share a full-text version of this article with your friends and colleagues. Every year, about … Ondine's or Undine's curse is a very rare medical condition characterized by respiratory arrest during sleep. Ondine’s curse — or, more officially, central hypoventilation syndrome — is an often fatal respiratory disorder that occurs during sleep. Semantic Scholar is a free, AI-powered research tool for scientific literature, based at the Allen Institute for AI. This rare form of apnea may require a patient to be on a ventilator to ensure that the patient is able to breathe while sleeping. The nymph Ondine was an immortal water spirit who became human after falling in love for a man, marrying him, and having a baby. It is due to arterial smooth muscle contraction caused by irritation by by‐products of red blood cell lysis. In 1990, Bogosslavsky et al reported two cases of respiratory failure caused by dorsolateral infarction of the medulla oblongata [9]. Materials and methods: This is a report of 3 cases of LMS with Ondine's curse. 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. Idiopathic congenital central hypoventilation syndrome: diagnosis and management. 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