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1/11. Hypersomnia in Wilson's disease: an unusual symptom in an unusual case.

    Wilson's disease (WD) shows a wide heterogeneity in symptoms. In this case report we present hypersomnia as a symptom of WD. The male patient's complaints as fatigue, decreased level of concentration, and highly increased demand of sleeping started at his age of 21 years. No abnormality was found at physical examination. A moderate elevation in liver function tests was found, but all the other laboratory findings were within the normal range. The marked hypersomnia was verified by 24-h cassette EEG polisomnographic monitoring. No abnormality was found at physical examination. EEG, brain CT and MRI were normal. Neither toxic nor infectious disease was detectable. The diagnosis of WD was based on decreased coeruloplasmin level, increased baseline and forced urinary excretion of copper, and decreased level of serum copper. Kayser-Fleischer ring was not detectable. D-penicillamine (DPA) was introduced. At 8-10 months after the initiation of the therapy the patient's complaints gradually resolved. The control sleep record 14 months after the initiation of the DPA therapy was normal. Five years later the patient is currently on penicillamine treatment and he is free of any symptom.
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2/11. 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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3/11. Excessive somnolence from aripiprazole in a child.

    A 9-year-old girl was hospitalized with prolonged somnolence that began 3.5 hours after a single 15-mg dose of aripiprazole. After extensive workup, physical examinations were unremarkable, and all laboratory test results were within normal limits. The patient's extreme somnolence was attributed to aripiprazole, a new atypical antipsychotic prescribed for oppositional defiant disorder. In clinical trials, somnolence has been reported with a frequency of approximately 11%. However, the somnolence experienced by this child was severe, requiring 24-hour hospitalization for observation. Although a recent dose-finding study in 22 pediatric patients (> or = 6 yrs old) described weight-based dosages that were safe and well tolerated, dosing of aripiprazole in children has not been well established. Further studies are needed to identify optimal dosing of aripiprazole in pediatric patients.
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4/11. Case study: psychiatric misdiagnosis of non-24-hours sleep-wake schedule disorder resolved by melatonin.

    This case study describes a 14-year-old male suffering from significant academic and personal difficulties, who has been diagnosed with depression, schizotypal personality disorder, and learning disabilities. Because of excessive sleepiness, assessment for a potential sleep disorder was performed. An overnight polysomnographic study revealed no primary sleep disorders. wrist actigraphy revealed a non-24-hour sleep-wake pattern. Delay in temperature rhythm and dissociation with melatonin rhythms were also noted. Treatment with oral melatonin restored normal sleep-wake schedule. In a follow-up psychiatric evaluation, none of the above diagnoses were present. Greater awareness of sleep disorders may prevent psychiatric misdiagnosis of treatable sleep-wake schedule disorders.
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5/11. Evaluating the sleepy patient.

    Excessive daytime sleepiness has various causes, and appropriate treatment varies accordingly. The diagnosis requires a history, physical examination, and polysomnographic study. A case report exemplifies the clinical approach.
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6/11. kleine-levin syndrome ethiopathogenesis and treatment.

    The complex of the symptoms of psychic disorders and of the disorders of sleep, appetite, and food intake often forms the basis of the clinical picture of a mental disease. However, it is only rarely conceived in a complex manner as a set of physiologically interdependent functions. A remarkable proof of the interdependence of these functions is their complex disorder, the kleine-levin syndrome. The first descriptions of the symptoms of the kleine-levin syndrome can be found in the studies of several authors published as early as at the turn of the century. In 1942, the syndrome was designated by Critchley and Hoffmann after Willi Kleine and Max Levin, who defined it precisely in 1925 and 1929. The syndrome of periodic hypersomnia, megaphagia, and psychic disorders, originally described only in young males, was later found in females as well; the original very strict criteria were gradually broadened and complemented to some extent. At present, the most commonly accepted criterion for the diagnosis of the kleine-levin syndrome is the existence of the combined sleep disorder (hypersomnia or insomnia lasting from days to weeks), food intake disorders (megaphagia or anorexia), and various psychic abnormalities accompanying or following the attacks of the affection. We term the syndrome typical if the sleep disorder appears in the form of hypersomnia, food disorder in the form of megaphagia, and if psychic abnormalities are clearly expressed. On the other hand, we term the syndrome atypical if one of the main symptoms is opposite. The incomplete syndrome consists of only two main symptoms. The attacks of the affection set on mostly suddenly, lasting from several days to several weeks, ending suddenly again. The interparoxysmal periods last from several days to several months, sometimes even to several years. The etiopathogenesis of the affection is still unknown. A number of reports indicate a disorder of the diencephalon, perhaps only of the hypothalamus. The pathological-anatomical findings following the death of persons suffering from the disorders of sleep and food intake and from psychic abnormalities mostly reveal lesions in the region of the third brain ventricle. The development of the typical syndrome is benign, however, and morphological studies are not available. The typical kleine-levin syndrome can hardly escape the attention of clinicians owing to the richness and clarity of symptoms. The atypical or discretely expressed forms, however, often remain unrecognized even after a detailed medical examination and may lead to diagnostic uncertainty.(ABSTRACT TRUNCATED AT 400 WORDS)
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7/11. kleine-levin syndrome with periodic apnea during hypersomnic stages--E.E.G. study.

    A 33 year old male, suffering from Kleine-Levine syndrome associated with periods of apnea during the hypersomnic attacks, is reported. Ventilatory studies negate the Pickwickian syndrome. The E.E.G.'s recorded during the hypersomnic attacks and the apneic periods showed a direct correlation between high-voltage delta waves paroxysmal E.E.G. activity, and apneic period. Medications known to improve kleine-levin syndrome, in our case, had no effect upon the clinical hypersomnic and apnea periods, nor on the correlatives E.E.G.'s pattern and spirometric studies. Theoretical considerations let us assume that these paroxysmal E.E.G. patterns associated with apnea are NRem-sleep serotonin dependent, and have an inhibitory influence on the respiratory centers, by alternating the equilibrium between the catecholamines and acetylcholine activities.
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8/11. Prominent somnolence and cerebellar syndrome in subclavian artery thrombosis--a case report.

    A patient suffering from acute left subclavian artery thrombosis presented with an unusual syndrome of prominent somnolence, right cerebellar signs, and left upper extremity ischemia. Clinical, angiologic, and electrophysiologic studies confirmed a brain stem and reticular formation dysfunction due to vertebrobasilar ischemia. Urgent thromboembolectomy led to neurologic improvement and satisfactory revascularization of the involved upper limb.
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9/11. kleine-levin syndrome: a case report.

    The case of a 10 yr old boy suffering from kleine-levin syndrome is described. The diagnosis was originally obscured by symptoms which suggested a primary depressive disorder. Unusual features included bizarre crying behaviour and unfounded allegations of sexual assault. Although the syndrome is rare, the need to be aware of the diagnosis is stressed, particularly in pre-pubertal children suffering from significant episodic depressive symptoms.
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10/11. Recurrent hypothermia, hypersomnolence, central sleep apnea, hypodipsia, hypernatremia, hypothyroidism, hyperprolactinemia and growth hormone deficiency in a boy--treatment with clomipramine.

    A boy referred at the age of 4 years because of obesity and under observation for 16 years, was found to be suffering from a hypothalamic syndrome of unknown origin characterized by progressive obesity, polyphagia, deficiency of growth and thyroid hormone, hyperprolactinemia, hypodipsia, hypernatremia and hyperosmolality without diabetes insipidus. At ages 11 and 16 there were 3 day episodes of spontaneous muscular weakness, hypersomnolence and hypothermia associated with central sleep apnea and severe bradycardia. Subsequently, decreased ventilatory responsiveness to carbon dioxide (CO2) was found as a consequence of blunted neural drive. Therapy with clomipramine HCl (Anafranil Ciba-Geigy) for 6 months led to a normalization of serum sodium levels, pulse rate, ventilatory response to dioxide with no recurrence of the central apnea within 4 following years.
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