1/15. Two cases of HLA-DR2-negative hypersomnia manifesting sleep-onset rapid eye movement periods in the multiple sleep latency test.We encountered two cases expressing excessive daytime sleepiness (EDS) and manifesting two or more sleep-onset rapid eye movement (REM) periods in the multiple sleep latency test. Unbearable daytime sleepiness occurred abruptly, which usually led to short-lasting naps, after which the patients felt refreshed. The EDS was successfully reduced by treatment with methylphenidate. In spite of these features similar to narcolepsy, these cases of REM hypersomnia did not present cataplexy or other auxiliary symptoms of narcolepsy, and, furthermore, the class-II human leukocyte antigen DR2 appeared to be negative.- - - - - - - - - - ranking = 1keywords = hypersomnia (Clic here for more details about this article) |
2/15. narcolepsy and other non-SAS hypersomnia in sleep breathing disorders clinic.Four of the 708 snorers (0.56%), referred to our sleep breathing disorders clinic for the past 2 years were diagnosed as having narcolepsy-cataplexy. Detecting HLA DRB1*1501/DQB1*0602 positive was informative for differentiating genuine narcolepsy from non-sleep apnea syndrome (non-SAS) hypersomnia in our clinic. A non-SAS obese boy, diagnosed as having essential hypersomnia syndrome, was found to be HLA DRB1*1502/DQB1*0601 positive. His hypocretin concentration was 206 pg/mL in the cerebrospinal fluid.- - - - - - - - - - ranking = 1.2keywords = hypersomnia (Clic here for more details about this article) |
3/15. 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 = 0.4keywords = hypersomnia (Clic here for more details about this article) |
4/15. Clinical caveat: prior sleep deprivation can affect the MSLT for days.It is common practice to forcibly awaken patients from an all-night polysomnographic study prior to performance of a multiple sleep latency test (MSLT); either for reasons of protocol or for the convenience of the laboratory personnel. We report a case of a young woman who, by formal sleep study criteria, received the erroneous diagnosis of narcolepsy due to the effects of severe sleep deprivation, and document that the effects of prior sleep deprivation require more than one night of ad libitum sleep. Forced awakening prior to MSLT may permit sleep deprivation or delayed sleep phase syndrome to masquerade as narcolepsy or idiopathic central nervous system (CNS) hypersomnia.- - - - - - - - - - ranking = 0.2keywords = hypersomnia (Clic here for more details about this article) |
5/15. 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 = 0.2keywords = hypersomnia (Clic here for more details about this article) |
6/15. narcolepsy caused by acute disseminated encephalomyelitis.BACKGROUND: narcolepsy with cataplexy is caused by a selective loss of hypocretin-producing neurons, but narcolepsy can also result from hypothalamic and rostral brainstem lesions. PATIENT: We describe a 38-year-old woman with severe daytime sleepiness, internuclear ophthalmoplegia, and bilateral delayed visual evoked potentials. Her multiple sleep latency test results demonstrated short sleep latencies and 4 sleep-onset rapid eye movement sleep periods, and her cerebrospinal fluid contained a low concentration of hypocretin. magnetic resonance imaging showed T2 and fluid-attenuated inversion recovery hyperintensity along the walls of the third ventricle and aqueduct, which are consistent with acute disseminated encephalomyelitis. RESULTS: After treatment with steroids, this patient's subjective sleepiness, hypersomnia, and hypocretin deficiency partially improved. CONCLUSIONS: autoimmune diseases such as acute disseminated encephalomyelitis can produce narcolepsy. Most likely, this narcolepsy is a consequence of demyelination and dysfunction of hypocretin pathways, but direct injury to the hypocretin neurons may also occur.- - - - - - - - - - ranking = 0.2keywords = hypersomnia (Clic here for more details about this article) |
7/15. Excessive daytime sleepiness in a trainee military pilot.A 21-yr-old army trainee pilot was noted by his colleagues to be frequently falling asleep in his class and was reported to medical authorities. Despite a number of investigations and review by two sleep specialists, a formal diagnosis could not be made. During his medical review board three questions were posed: does the trainee have excessive daytime sleepiness (EDS), or is his sleepiness a variation of normal; if he is excessively sleepy, what is the most likely diagnosis; and should the trainee be allowed to continue to undertake pilot training based on most likely diagnosis and/or his history. The most likely diagnosis was considered to be idiopathic hypersomnia, a relatively uncommon condition which is a diagnosis of exclusion. The condition was considered incompatible with flight duties in a pilot under training due to the potential for compromise of performance, mission completion, and flight safety. Stimulant medications may control symptoms, but are incompatible with flying duties in the Australian Defense Force. This case illustrates the difficulties in determining aeromedical disposition in borderline cases of EDS where a clear diagnosis cannot be made.- - - - - - - - - - ranking = 0.2keywords = hypersomnia (Clic here for more details about this article) |
8/15. 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 = 1keywords = hypersomnia (Clic here for more details about this article) |
9/15. 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 = 0.2keywords = hypersomnia (Clic here for more details about this article) |
10/15. Marked blood pressure fluctuations during narcoleptic attacks alternating with abnormal wakefulness: effects of treatment with clonidine.A middle-aged man was admitted to our department because of sleep-wake cycle disorders (alternating hypersomnia and sleeplessness), bipolar behavioural disturbances and marked fluctuations in blood pressure and heart rate. Neither evident precipitating stimuli nor an obvious cause for his illness were found. When tests that normally activate intrinsic autonomic responses were performed, two distinct circulatory patterns were recognized. During hypersomnia (phase A), cardiovascular reflex activity was blunted or abolished and orthostasis could not be maintained. The clinical, biochemical, behavioural pictures and the observed decrease in sympathetic outflow resembled the effects of clonidine administration. On the contrary, during sleeplessness (phase B) the autonomic pathways were functionally integral and orthostatic hypotension was not detected. The clinical, biochemical, behavioural features and cardiovascular overactivity closely mimicked the abrupt withdrawal syndrome encountered with clonidine. Three hypothetical mechanisms are advanced to explain this intriguing case as well as the acute and chronic relief of our patient's clinical problem following institution of clonidine therapy (phase C). The role played by central alpha adrenoceptors in integrating sleep-wake, cardiovascular and behavioural functions is also suggested.- - - - - - - - - - ranking = 0.4keywords = hypersomnia (Clic here for more details about this article) |
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