1/12. Kleine-Levin and Munchausen syndromes in a patient with recurrent acromegaly.Hypothalamic disease often affects the patients' personality and this also applies to pituitary tumors with suprasellar extension. We report on a patient with a 12-year history of recurrent acromegaly, treated with three transphenoidal operations, single field radiation therapy and bromocriptine/octreotide administration. During the course of follow-up she presented with self-inflicted anemia and kleine-levin syndrome (hypersomnia, hyperphagia and hypersexuality). Furthermore, she developed post-radiation necrosis within the right temporal lobe. Whether her neurological and personality disorders result - at least partially - from the acromegaly or the temporal lobe necrosis remains unclear.- - - - - - - - - - ranking = 1keywords = hypersomnia (Clic here for more details about this article) |
2/12. The kleine-levin syndrome as a neuropsychiatric disorder: a case report.The kleine-levin syndrome (KLS) is characterized by periodic, sudden-onset episodes of hypersomnia, compulsive hyperphagia, and behavioral-emotional disorders (typically indiscriminate hypersexuality, irritability, impulsive behaviors), lasting from a few days to a few weeks, with almost complete remission in the intercritical periods. depression, confusion, and thought disorders are frequently associated with the critical symptomatology, and they may suggest other psychiatric diagnoses (schizophrenia, mood disorder, conversion disorder) or a substance abuse. A diencephalic-hypothalamic dysfunction is suspected, even if this composite symptomatology cannot easily be linked to a simple mechanism. The aim of this article is to illustrate problems in differential diagnosis, using a case approach. History, course, and therapeutic intervention in a 21-year-old patient with KLS, associated with a clear psychiatric symptomatology and a critical affective pattern, is reported. Psychiatric correlates of KLS are discussed, including the relationship with affective disorders and the possible emotional impact of the attacks. Implications regarding a combined psychological and pharmacological treatment are also discussed.- - - - - - - - - - ranking = 1keywords = hypersomnia (Clic here for more details about this article) |
3/12. Neuropsychological sequelae in kleine-levin syndrome: case report.kleine-levin syndrome is characterized by periodic hypersomnia, hyperphagia, sexual disinhibitions and behavioral disturbances. The prognosis is generally benign, with normal cognitive and social functions after the episodes. We describe a typical case of kleine-levin syndrome associated with apparent academic decline, neuropsychological sequelae and personality alterations after the second episode of the illness. Further research in the natural history of kleine-levin syndrome is needed, for example, to determine whether early intervention would improve long-term prognosis.- - - - - - - - - - ranking = 1keywords = hypersomnia (Clic here for more details about this article) |
4/12. The kleine-levin syndrome. Report of a case and review of the literature.kleine-levin syndrome is a rare self-limited disorder which usually affects adolescent males and is characterized by episodic hypersomnia, increased appetite, and behavioral/psychiatric disturbances. Individuals are normal between the attacks. The case of an adolescent boy is presented who suffered from recurrent sleepiness, hyperphagia, and behavioral disturbances such as rocking, punching and pacing, and was originally misdiagnosed as suffering from encephalitis. Before the diagnosis of Kleine-Levin was given, the patient underwent unnecessary investigations and treatment which, in turn, complicated his clinical condition both physically as well as psychologically. In the course of five years he had four such episodes which appeared to have progressively milder manifestations. Between episodes he was normal. It is important that the diagnosis is suspected early, especially in adolescent males who present with recurrent episodes of somnolence, increased appetite, and abnormal behavior, since it most often represents a benign and self-limited entity and does not warrant extensive investigations or treatment. It is also important to distinguish this syndrome from more serious organic and psychiatric diseases with more serious prognoses. The differential diagnosis of this syndrome is discussed and a review of the literature is presented including evidence and hypotheses regarding its pathophysiology.- - - - - - - - - - ranking = 1keywords = hypersomnia (Clic here for more details about this article) |
5/12. Multiple sleep latency test and polysomnography in diagnosing kleine-levin syndrome and periodic hypersomnia.kleine-levin syndrome and periodic hypersomnia are often misdiagnosed initially because there is no objective test for these conditions. To determine the value of the Multiple sleep Latency Test and polysomnography in this respect, the authors studied four patients with kleine-levin syndrome or periodic hypersomnia who had taken the Multiple sleep Latency Test and undergone polysomnography during the symptomatic episode and/or during the asymptomatic interval. During but not between symptomatic episodes, the Multiple sleep Latency Test revealed abnormal sleep latencies in all patients, and polysomnography revealed increased rapid eye movement propensity in one patient and a reduction in delta-sleep in two patients. In conclusion, the Multiple sleep Latency Test and polysomnography are useful in diagnosing kleine-levin syndrome and periodic hypersomnia, especially when administered in a standardized fashion during and after the symptomatic period. The authors recommend that polysomnography and the Multiple sleep Latency Test be performed no earlier than the second night after the onset of a symptomatic episode and the following day to reveal maximal hypersomnolence, and more than 2 weeks after a symptomatic episode to represent the asymptomatic interval.- - - - - - - - - - ranking = 7keywords = hypersomnia (Clic here for more details about this article) |
6/12. A case of kleine-levin syndrome examined with SPECT and neuropsychological testing.A case of kleine-levin syndrome with typical periodic hypersomnia and bulimia was diagnosed. On examination with single photo emission tomography (SPECT) (CERETEC) during a relapse period and 2 weeks later there was marked cortical hypoperfusion of the frontal and temporal lobes, especially on the left side as well as in the right parietal lobe. Neuropsychological testing performed 1 week after a relapse showed a reduction in encoding to memory function of verbal learning indicating neocortical damage of the left fronto-temporal region. A follow-up 2 months later after the patient had spontaneously recovered showed only a slight left fronto-temporal disturbance. CT and MRI of the brain were normal although the MRI showed a large and asymmetric mamillary body. Neuropsychological testing 6 years after recovery showed pronounced reduction in short-time verbal and visual memory. Seven years after recovery SPECT demonstrated a normalized frontal perfusion but still a slight hypoperfusion in the left temporal lobe. Our results correlate to autopsy findings in two cases described previously.- - - - - - - - - - ranking = 1keywords = hypersomnia (Clic here for more details about this article) |
7/12. The kleine-levin syndrome - effects of treatment with lithium -.kleine-levin syndrome (KLS) is a rare disorder which affects mainly adolescents. Periods of extreme somnolence alternate with megaphagia, psychomental changes and behavioural symptoms. The cause and pathogenesis of KLS remains unknown. Several treatments have been tried and recently lithium has been proposed for a prophylactic use in single cases. In view of the rarity of KLS, long-term results of lithium therapy have not been described yet. We report the clinical course of five adolescents with KLS who were treated with lithium. All patients showed significant EEG and polysomnographic changes during the episodes and had normal results in the interval. All patients had relapses while being treated with lithium. But episodes of hypersomnia under lithium therapy were shorter and monosymptomatic with lack of behavioural symptoms. Statistical modelling showed that the risk for a relapsing episode under maintenance of lithium drops per months of therapy from 100 % to 93 %, and furthermore that the maintenance of lithium shortens the mean duration of episodes to 19 %. No severe side effects were observed. In conclusion, in KLS with a high frequency of episodes and severe behavioural changes lithium may become a treatment option.- - - - - - - - - - ranking = 1keywords = hypersomnia (Clic here for more details about this article) |
8/12. Episodic diencephalic hypoperfusion in kleine-levin syndrome.A 22 year-old woman suffered from recurrent episodes of hypersomnia, apathy, and hyperphagia. The symptoms occurred 3 to 4 times per year, and each attack lasted 2 to 3 weeks. 99mTc-ethylcysteinate dimer brain single photon emission computed tomography (SPECT) was performed during symptomatic and asymptomatic periods. To localize brain regions with perfusion changes during symptomatic period, asymptomatic SPECT was subtracted from symptomatic SPECT. The subtracted SPECT showed significant hypoperfusion in the left hypothalamus, bilateral thalami, basal ganglia, bilateral medial and dorsolateral frontal regions, and left temporal lobe during the symptomatic period. These cerebral hypoperfusion areas support the diencephalic hypothesis and clinical symptoms of kleine-levin syndrome.- - - - - - - - - - ranking = 1keywords = hypersomnia (Clic here for more details about this article) |
9/12. Electrophysiological and immunogenetic findings in recurrent monosymptomatic-type hypersomnia: a study of two unrelated Italian cases.MSLT and immunogenetic findings in two unrelated Italian subjects with recurrent monosymptomatic hypersomnia are reported. In both patients MSLT documented a markedly increased daytime sleep propensity during the attacks without augmented REM sleep pressure. Both patients share the same HLA haplotype (HLA-DR1, DQ1) which has been found in kleine-levin syndrome. This makes these subtypes of recurrent hypersomnia indistinguishable one from the other, under the immunogenetic profile, but permits differentiation from narcolepsy which is HLA-DR2, DQ1 closely linked.- - - - - - - - - - ranking = 6keywords = hypersomnia (Clic here for more details about this article) |
10/12. kleine-levin syndrome in a boy with prader-willi syndrome.A 9 1/2-year-old Taiwanese boy with prader-willi syndrome had the following characteristics: difficulties with sucking, feeding and hypotonia during infancy, a dysmorphic face (triangular mouth, high arched palate, almond-shaped eyes and large head circumference with a relatively narrow bifrontal diameter), borderline intelligence, hypogonadism, hyperphagia, skin picking and truncal obesity. The boy experienced two hypersomnia episodes, at age 8 and 9 years, with both episodes lasting for 10 days. During the two episodes, he was found to have an exacerbated case of hyperphagia, pica, poor emotional control, stereotyped speech and agitated behavior upon awakening. After each episode, the boy had complete remission. Our findings show that the two episodes are compatible with kleine-levin syndrome. The relationship between the two syndromes, the prader-willi syndrome and the kleine-levin syndrome, deserves further study.- - - - - - - - - - ranking = 1keywords = hypersomnia (Clic here for more details about this article) |
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