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1/8. 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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2/8. Postoperative ventilatory failure in an obese, myopathic woman with periodic somnolence: A case report.

    An obese woman with periodic sommolence developed postoperative ventilatory problems following an uncomplicated cholecystectomy. A severe myopathic process involving the respiratory muscles was found at necropsy. A brother, who died a week later, had myotonic dystrophy.
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keywords = process
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3/8. methylphenidate hydrochloride for excessive daytime sleepiness in a patient with myotonic dystrophy.

    A 28-year-old man with a history of myotonic dystrophy (MyD) is described. His progressively worsening excessive daytime sleepiness (EDS) was treated with methylphenidate hydrochloride. Polysomonography (PSG), the multiple sleep latency test (MSLT), middle latency-evoked response, and auditory event-related potentials were used to assess the cause of EDS. The PSG detected small numbers of central sleep apneic episodes. Mean sleep latency, which was determined by MSLT, increased from 5 min before treatment to 14 min after treatment. The Pa, P1, and N1 amplitudes at the Cz site increased after treatment, but P3 latency remained unchanged. These observations suggest that EDS and impairment of cognitive and information processing seen in a patient with MyD may be caused by a central nervous system disorder.
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keywords = process
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4/8. sleep disorders.

    humans spend approximately one third of their lives asleep. Although the same medical disorders that occur during wakefulness persist into sleep, there are many disorders that occur exclusively during sleep or are manifestations of a disturbance of normal sleep-wake physiology. The most common reason for referral to a sleep laboratory is OSA, whereas the most common sleep disorder is insomnia. Effective treatments now exist for many sleep disorders, such as OSA and RLS, and a major breakthrough in the treatment of narcolepsy seems imminent. Because all disease processes are adversely affected by insufficient sleep, it is essential that the practicing physician understand the causes and treatments of the common sleep disorders.
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ranking = 0.0020623264035014
keywords = process
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5/8. Alveolar hypoventilation and hyperosmnia in myotonic dystrophy.

    A case of myotonic dystrophy accompanied by alveolar hypoventilation and hypersomnia is presented. Radiological studies and EMG examination of the intercostal muscles demonstrated that the respiratory muscles were affected by the disease, while polygraphic recordings showed that the alveolar hypoventilation and pulmonary hypertension worsened during sleep. The hypersomnia preceded the appearance of clinical signs of the muscular disease by many years and persisted even after treatment when the blood gas analysis values were greatly improved. During both diurnal and nocturnal sleep, the patient frequently fell asleep directly into a REM stage. The possibility is discussed that, concomitant with the respiratory musculature involvement, there is an alteration in the central nervous system in myotonic dystrophy which is at least partially responsible for both the alveolar hypoventilation and the hypersomnia.
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6/8. 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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7/8. myotonic dystrophy with alveolar hypoventilation and hypersomnia: a clinicopathological study.

    We present a case of myotonic dystrophy accompanied by alveolar hypoventilation and hypersomnia. Case history, pulmonary function tests, polygraphic recording, and multiple sleep latency test, concomitant with a restrictive ventilatory abnormality, suggested a central origin of alveolar hypoventilation and hypersomnia in our case. The most significant neuropathological findings were in the tegmentum of the brain stem. Severe neuronal loss and gliosis were observed in the midbrain and pontine raphe, particularly in dorsal raphe nucleus and superior central nucleus. Pontine and medullary reticular formation also showed a marked cell loss and fibrillary gliosis. The alveolar hypoventilation and the hypersomnia in our case may be attributed to these morphological abnormalities, and would appear to be central in nature.
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keywords = alveolar
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8/8. 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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