1/6. Decreased cerebrospinal fluid hypocretin-1 levels near the onset of narcolepsy in 2 prepubertal children.We present 2 cases of narcolepsy with prepubertal onset. Although excessive daytime sleepiness and cataplexy had appeared early in both patients, the presence of sleep-onset rapid eye movement periods was detected several months after the onset of hypersomnia. The levels of hypocretin in the cerebrospinal fluid were reduced when measured 3 weeks (Patient 1) and 2 months (Patient 2) after the appearance of hypersomnia, before the presence of sleep-onset rapid eye movement periods was confirmed. Because the symptoms of narcolepsy in children are often obscure and easily mistaken as other diseases, and the electrophysiologic studies may not be specific in the early stage, the definite diagnosis tends to be delayed. Measurement of hypocretin-1 levels in the cerebrospinal fluid is useful for the early diagnosis of narcolepsy with prepubertal onset.- - - - - - - - - - ranking = 1keywords = hypersomnia (Clic here for more details about this article) |
2/6. sleep disorders.patients with sleep disorders present with a variety of complaints including excessive daytime sleepiness, daytime spells, inability to sleep, uncomfortable sensation in the extremities, and unusual night time behaviors. This article provides eight vignettes on patients with sleep disorders including narcolepsy, idiopathic hypersomnia, obstructive sleep apnea, restless legs syndrome, and rapid eye movement behavior disorder. The discussion provides data regarding the epidemiology, pathophysiology, and diagnostic approach for these conditions. The various treatment options for these sleep disorders are also identified.- - - - - - - - - - ranking = 0.5keywords = hypersomnia (Clic here for more details about this article) |
3/6. protriptyline: an effective agent in the treatment of the narcolepsy-cataplexy syndrome and hypersomnia.The authors present five case reports illustrating that 10-20 mg of protriptyline in a single dose at bedtime can effectively control arousal dysfunction (sleep drunkenness and hypersomnia) and the narcolepsycataplexy syndrome without the apparent development of tolerance and without the side effects that are frequent complications of treatment with other agents. Although protriptyline was efficacious in controlling symptoms, it was found to have relatively poor REM sleep-suppressing properties.- - - - - - - - - - ranking = 2.5keywords = hypersomnia (Clic here for more details about this article) |
4/6. Characteristics of narcolepsy in preteenaged children.narcolepsy is rarely diagnosed in preteenaged children. Its clinical and polysomnographic manifestations, some of which are unusual, are described in four children who were observed prospectively. The mean age at onset of hypersomnia was 10.2 years (range 8.4 to 11.2 years). Daytime naps among these children were lengthy, ranging from 20 to 120 minutes, and generally were considered unrefreshing. cataplexy was present at the onset in all four children. Three of the four children were obese, with the concurrent nocturnal snoring prompting a misleading concern about obstructive sleep apnea syndrome in two children. The histocompatibility DR2 antigen was present in all four children. Significant behavioral manifestations appeared in all of them. The response to stimulant medications was, at best, modest. narcolepsy may be difficult to diagnose in this age group. However, a careful history eliciting sleep/wake dysfunction (including cataplexy), leukocyte assays for the histocompatibility DR2 antigen, and serial polysomnographic studies may enable early recognition and treatment of this disease.- - - - - - - - - - ranking = 0.5keywords = hypersomnia (Clic here for more details about this article) |
5/6. The pathophysiology of sleep disorders in pediatrics. Part II. sleep disorders in children.In this part of the chapter we have used new terminology and developed a new system for classification of sleep disorders in children. We suggest that excessive daytime sleepiness should be investigated by clinicians before troubles at school necessitate referral. The narcolepsy-hypersomnia syndrome generally has not been recognized in the pediatric age group. Symptoms of excessive fear of falling asleep need to be viewed in this context. Sleep apnea-hypersomnia has received insufficient attention in the American literature. It is a syndrome that affects both adults and children with potentially disastrous cardiovascular and pulmonary complications. The relationship of the sleep apnea-hypersomnia syndrome to the sudded infant death syndrome remains speculative, although preliminary results from our longitudinal study have indicated a possible link. Both the narcolepsy-hypersomnia and the sleep apnea-hypersomnia syndromes are reviewed in detail. In contrast, we review briefly the NREM dyssomnias, including night terrors, sleepwalking, sleep talking and enuresis. All are well known to clinicians dealing with children, and we have related them to findings emanating from the sleep laboratory. We suggest that they are physiologically rather than psychogenically based and frequently represent immaturities of the central nervous system. Finally, the insomnias of childhood are presented. We emphasize that they are rare, and after ruling out organic conditions and drug-dependency syndromes, cultural styles or family stresses generally account for the majority of complaints.- - - - - - - - - - ranking = 2.5keywords = hypersomnia (Clic here for more details about this article) |
6/6. Fibrositis syndrome and narcolepsy.Fibrositis is often associated with sleep disturbances and with an alpha nREM abnormality on sleep electroencephalogram. We describe a case occurring during the course of a typical longstanding narcolepsy-cataplexy. Modafinil, that is an effective treatment of hypersomnia, did not alleviate the symptoms of fibrositis in the short term.- - - - - - - - - - ranking = 0.5keywords = hypersomnia (Clic here for more details about this article) |