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Query: UMLS:C0037315 (sleep apnea)
 8,000 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We have described a 13-year-old white boy with Ondine's curse, sleep apnea and cataplexy who simultaneously developed progressive hypothalamic neuroendocrine deficiencies requiring replacement therapy. The patient was treated with protriptyline, a nonsedating tricyclic antidepressant, for control of the sleep-related symptoms. An unexpected result was the apparent reversal of his chronic diabetes insipidus by protriptyline, necessitating discontinuation of lysine-vasopressin. Some possible mechanisms of action were discussed.
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PMID:Reversal of chronic diabetes insipidus during treatment with protriptyline. 72 36

Hirschsprung's disease can be associated with other congenital abnormalities, some of which are neural in origin. A rare association is with congenital failure of automatic control of respiration--central hypoventilation syndrome, sleep apnoea or Ondine's curse. Patients with this combination tend to have a short life expectancy. Diaphragmatic pacing by electrophrenic stimulation has proven useful in management of patients with central hypoventilation. Three children, two females and one male, with this combination are described. The male child, who had total intestinal aganglionosis, died at the age of one month despite an ileostomy and nocturnal mechanical ventilation. The two females had aganglionosis more typical of Hirschsprung's disease, one requiring colostomy. At the ages of 2 and 6 years, respectively, phrenic nerve stimulators were implanted. Both girls remain independent of nocturnal, mechanical ventilation two and three years after commencement of diaphragm pacing. In patients with Ondine's curse and Hirschsprung's disease in whom the aganglionosis can be effectively managed, diaphragm pacing may lead to independence from mechanical ventilation and prolongation of life of an acceptable quality.
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PMID:Ondine's curse with Hirschsprung's disease. 235 89

Thirty-five patients, 21 males and 14 females, aged 2-69 years (mean 25 years) with partial or total respiratory paralysis due to high cervical cord lesions, brain stem lesions, or central hypoventilation syndrome (sleep apnoea, 'Ondine's curse'), received phrenic nerve stimulators for pacing of the diaphragm. At a mean follow-up time of 46 months (range 2 months to 10 years) 15 patients are entirely independent of respirator and 8 quadriplegics ventilate with pacers at different daytime intervals and use mechanical ventilators during the night. Five patients have stopped pacing and 7 additional cases have died of causes unrelated to electrophrenic stimulation.
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PMID:Pacing of the diaphragm to control breathing in patients with paralysis of central nervous system origin. 251 91

Sleep-induced narrowing of the upper airways underlies the widespread and supposedly trivial complaint of snoring, which may not only constitute a risk factor for the cardiocirculatory system, but in predisposed individuals may lead to the OSAS. The latter is a life-threatening condition characterized by repeated episodes of cessation of respiration at night with an associated drop in SaO2. Patients frequently present with hypersomnia, systemic and pulmonary hypertension, and even heart failure. HSD is the term we use to describe the evolutive stages from snoring to OSAS. ICAH, or Ondine's curse, is the clinical syndrome of sleep-related respiratory insufficiency in the absence of airway stenosis. We do not consider central sleep apnea to be an independent disorder. For the treatment of HSD, weight reduction should be attempted first. Also, if there are malformations in the upper airway, they should be surgically corrected. The use of various medications has been rather discouraging, and CPAP and other devices that are intended to overcome the obstruction are poorly tolerated by patients. The most effective surgical treatment for OSAS, even in progressed stages of the disease, is tracheostomy.
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PMID:Sleep-related respiratory disorders. 333 61

Primary alveolar hypoventilation is a rare syndrome of unknown origin, characterized by a dysfunction of the automatic respiratory pattern in spite of normal lungs and in the absence of mechanical ventilatory defects. A reduction of the ventilatory response to CO2 is regularly found, and cardiac failure is common. The differential diagnosis mainly concerns the sleep apnoea syndrome. The usual treatments have little effectiveness.
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PMID:[Primary alveolar hypoventilation. Clinical aspects and diagnostic problems. Apropos of a case]. 367 71

Central sleep apnea is a disorder characterized by apneic episodes during sleep with no associated ventilatory effort. More commonly than not these apneas are seen in patients who also have obstructive and mixed events. Although patients with this disorder frequently complain of insomnia and depression, frank hypersomnolence is rarely encountered. As these complaints are common ones seen in numerous clinical situations, and since sleep studies are rarely conducted to investigate their etiology, the true incidence of central sleep apnea has not been determined. The etiology of central apnea remains unknown, although the association between these breathing events and a number of other disease processes has increased our understanding of the disorder. Central apneas during sleep commonly occur after hyperventilation with the associated hypocapnic alkalosis. This occurs at high altitude when hyperventilation is induced by hypoxia and at sea level when spontaneous nocturnal hyperventilation occurs. This suggests that PCO2 is the primary stimulus to ventilation during sleep and that loss of this drive, as occurs with hypocapnia, may produce dysrhythmic breathing. Patients with complete absence of ventilatory chemosensitivity such as occurs with Ondine's curse (central alveolar hypoventilation) or the obesity-hypoventilation syndrome may also have central apneas. For reasons that remain unexplained, central sleep apnea is commonly seen in patients with congestive heart failure, nasal obstruction, and certain neurologic disorders. However, in most patients with central sleep apnea no obvious cause or association can be found. The treatment of this disorder is not entirely satisfactory. If it is severe, mechanical ventilation during sleep can be provided by any one of a number of techniques. However, for the patient who simply complains of insomnia and is found to have a moderate number of central apneas, the treatment choices are limited. Acetazolamide has been shown to decrease central apneas during short-term use, but results have been variable with prolonged administration. Other ventilatory stimulants seem to have little efficacy. Interestingly, oxygen administration has been shown to reduce central apneas considerably in a number of studies, although the explanation for its success is unknown. Central sleep apnea therefore remains a relatively rare disorder whose etiology is not fully understood and whose treatment is not completely satisfactory.
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PMID:Central sleep apnea. 393 82

Neurologists are becoming increasingly aware of the frequency and clinical importance of sleep-related respiratory impairment. Sleep-induced narrowing of the upper airways underlies the widespread and supposedly trivial complaint of snoring, which may not only constitute a risk factor for the cardiocirculatory system, but in predisposed individuals, may lead to a sleep apnea syndrome, with its array of serious disturbances, including hypersomnia, systemic and pulmonary hypertension and ultimately heart failure. Idiopathic chronic alveolar hypoventilation, or Ondine's curse, is a fairly stereotyped clinical syndrome: sleep-related respiratory insufficiency in the absence of airways stenosis. Finally, sleep, and REM sleep in particular, significantly aggravates hypoventilation in patients with chronic obstructive pulmonary disease (COPD), kyphoscoliosis or chest musculoskeletal disorders.
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PMID:Sleep-related respiratory disorders. 408 59

We obtained the cardio-respiratory impulse response and noise contribution ratio to study the central respiratory control system in one case of Ondine's curse, one case of sleep apnea syndrome and 34 cases of preterm infants, using multivariate autoregressive analysis. In Ondine's curse, the noise contribution ratio decreased during sleep. In the sleep apnea syndrome, the noise contribution ratio before apnea and after apnea decreased. In the preterm infants, the noise contribution ratio increased with the conceptional age. From these results, we concluded that the central cardio-respiratory control system was disturbed in Ondine's curse, sleep apnea syndrome and preterm infants. The cardio-respiratory impulse response using a multiple autoregressive analysis was useful for prediction of sudden infant death syndrome.
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PMID:[Estimation of SIDS with measurement of cardio-respiratory contribution ratio through multivariate autoregressive modelling approach]. 883 Dec 39

This report describes a rare case of acquired Ondine's curse. The patient developed central sleep apnea syndrome named Ondine's curse after a brainstem infarction. Lesions involving the descending medullocervical pathways that subserve automatic breathing can result in this syndrome.
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PMID:Ondine's curse after brainstem infarction. 1943 58

Sleep is a biological mechanism essential to maintaining good health. A series of physiological changes takes place during sleep. From a ventilation point of view, during deep sleep, tidal volume increases, the inhalation and expiration phases become longer, and respiratory frequency slows, while the same volume/time ratio as in wakefulness is maintained. The concept of "sleep-related respiratory disorders" refers to a group of respiratory diseases that are aggravated during sleep. Some of these disorders are almost exclusively manifested during sleep, such as sleep apnea-hypopnea syndrome -undoubtedly the most frequent respiratory disease despite being underdiagnosed- and congenital central hypoventilation syndrome (also called Ondine's curse). However, this concept also encompasses other disorders that occur during the daytime since they worsen while patients are asleep, as seen in many obstructive or restrictive pulmonary diseases. Sleep disorders can be a manifestation of underlying diseases, some of which are highly prevalent such as obesity and gastroesophageal reflux. Likewise, abnormal sleep cycles may be a cause or a result of cognitive disorders and disturbances in children's learning processes.
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PMID:[Obstructive sleep-related respiratory disorders in children]. 2135 86


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