Cases reported "Sleep Disorders"

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1/12. Sleep disordered breathing during REM sleep in Freeman-Sheldon syndrome.

    OBJECTIVES: To examine the sleep-disordered breathing in patients with Freeman-Sheldon syndrome (FSS). MATERIAL AND methods: One night polysomnography was performed for 2 teenage FSS patients. RESULTS: They showed frequent obstructive sleep apnea exclusively during rapid-eye-movement sleep. CONCLUSION: FSS is a risk factor for the occurrence of sleep disordered breathing.
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2/12. Hypersomnia with periodic breathing (an acromegalic Pickwickian).

    The syndromes of Pickwickian, Ondine's curse, and primary alveolar hypoventilation are respiratory disorders manifesting increased sleepiness and irregular respiratory rhythms. These disorders are currently grouped as hypersomnia with periodic breathing (HPB). Polygraphic techniques have lead to a reasonable hypothesis as to the pathophysiology of the multiple variants of HPB. Discernible causes of HPB have been attributed to both central and peripheral factors. Peripheral factors encompass those conditions relating to upper airway obstruction. An acromegalic person suffering the HPB syndrome secondary to laryngeal stenosis is described.
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3/12. Pickwickian syndrome, 20 years later.

    The Pickwickian Syndrome stimulated new pathophysiological concepts in regard to control of ventilation. With the advent of sleep laboratories, the peculiar sleep apnea occurring in some of these patients has been explained on the basis of intermittent upper airway obstruction. Two patients with different manifestations of the Pickwickian Syndrome are presented. The suggestion is made that these two subsyndromes should have unique designations. The Auchincloss Syndrome is manifested by right heart failure and respiratory acidosis in obese patients who are alert and have no major abnormality of breathing pattern. The fundamental cause of this abnormality is the increased work of breathing caused by the obesity. The cost of breathing is so high that the ventilatory regulation is compromised and respiratory acidosis results. The Gastaut Syndrome is characterized principally by hypersomnia and sleep apnea. The fundamental defect is upper airway obstruction during sleep, resulting in increased work of breathing, which together with the increased work caused by obesity leads to respiratory acidosis and right ventricular failure. Hypersomnia, rather than heart failure or respiratory acidosis, is the major manifestation of this syndrome, and is the result of sleep loss.
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4/12. Sleep-related headache syndromes.

    The relationship between sleep and sleep disorders and headache remains unclear. Clinical experience and numerous studies document some sort of relationship, but the exact nature remains understudied and complex. Changes in sleep duration and sleep quality appear to be capable of affecting headaches of different types. Obstructive sleep apnea can cause or exacerbate headaches in a susceptible person. Obstructive sleep apnea also may cause a specific headache when awakening, which is different from migraine or tension headache and disappears after treatment of the sleep and breathing disturbance. Hypnic headache is another type of sleep-exclusive headache that has been proposed. Hypnic headaches are brief, moderately severe, and affect the elderly primarily.
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5/12. Sleep and breathing abnormalities in a case of prader-willi syndrome. The effects of acute continuous positive airway pressure treatment.

    This report describes the polysomnographic findings and the respiratory alterations during sleep in a 20-year-old patient with the prader-willi syndrome. Nocturnal recordings and a variant of the multiple sleep latency test showed excessive daytime sleepiness, sleep onset rapid eye movement episodes, snoring and sleep apnea. Treatment with nasal continuous positive airway pressure normalized the respiratory pattern and the sleep structure, except for rapid eye movement sleep onset. Whereas upper airway obstruction and obesity may explain the respiratory disorders, as shown by their resolution with continuous positive airway pressure treatment, hypothalamic dysfunction could play a role in the disruption of the normal nonrapid eye movement/rapid eye movement sleep periodicity.
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6/12. Hypersomnia and periodic breathing. Report of a case and review of the literature.

    A patient with hypersomnia and periodic breathing has been studied. Both airflow obstruction and an abnormally of the respiratory control mechanism were implicated in the pathogenesis of the ventilatory arrhythmia. It is suggested that the older terms "Pickwick" syndrome and primary alveolar hypoventilation are abandoned for more descriptive terms, e.g. "hypersomnia with periodic breathing".
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7/12. Further observations on sleep abnormalities in kleine-levin syndrome: abnormal breathing pattern during sleep.

    In two adolescent and two adult patients with kleine-levin syndrome, polygraphic sleep recording performed during somnolent and non-somnolent periods revealed various forms of abnormal breathing patterns during sleep. These included periodic breathing and hypopnoeic episodes associated with brief arousals and, in one adult patient, a full blown sleep apnoea syndrome. It is suggested that abnormal breathing in sleep in this syndrome may result from central hypoexcitability.
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8/12. Polygraphic study of periodic breathing and hypersomnolence in a patient with severe hypothyroidism.

    A 67-year-old woman with primary hypothyroidism, who had Pickwickian syndrome as well, was studied electrophysiologically. A polygraphic study with simultaneous recordings of electroencephalography, actograms of the abdominal walls, the mentum and the anterior neck, and electromyogram of the intercostal muscles revealed a cyclic appearance of apnea in the sleep phase and gasping preceding arousal, which, together with macroglossia and sleep in the sitting position, suggested a cyclic obstruction of the upper airway. The obstruction appeared responsible for both alveolar hypoventilation and disturbance of consciousness.
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9/12. Sleep-related breathing disorders in patients with multiple system atrophy and vocal fold palsy.

    We performed sleep studies in eight patients with multiple system atrophy (MSA) and three patients with peripheral bilateral vocal fold palsy (PBVFP) and investigated stenosis of the upper airway tract during sleep in MSA patients with vocal fold palsy. Among the eight MSA patients in this study, five had definite glottic snoring and two others were suspected of having glottic snoring. Of the PBVFP patients, two had glottic snoring. Three of 11 patients died, and two of the three deaths occurred during sleep. Glottic snoring indicated a high degree of negative esophageal pressure. High negative esophageal pressure demonstrates severe narrowing of the upper airway tract. Therefore, glottic snoring should be considered a risk factor for sudden death in sleep. Repeated laryngoscopic examination is useful in evaluating the progressive process of vocal fold palsy while awake, but this examination performed only while awake is not enough to evaluate narrowing of the upper airway during sleep. Sleep studies that include the measurement of esophageal pressure can be very useful in evaluating the severity of narrowing in the upper airway tract. It is suspected that sudden nocturnal death in MSA patients is caused not only by abnormal respiration resulting from impairment of the respiratory center, but also by glottic obstruction caused by sputum or by edema of the vocal folds. We recommend treatment of respiratory disorders when loud laryngeal snoring occurs in patients with MSA, even if they do not complain of dyspnea while awake.
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10/12. Paroxysmal nocturnal dyspnoea and periodic respiration.

    Patterns of breathing at night were recorded in 4 patients with heart-failure. 2 had periodic breathing while awake and in 2 it developed after they fell asleep. In all 4 the phase of hyperventilation disturbed sleep. These cases also illustrate other problems caused by periodic respiration in heart-failure, which range from tiredness during the day to an inability to sleep for more than a few minutes. Nocturnal waking in the hyperventilation phase of Cheyne-Stokes breathing should be differentiated from paroxysmal nocturnal dyspnoea caused by episodes of pulmonary oedema at night.
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