1/23. 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. ( info) |
| The kleine-levin syndrome is a rare disorder with its main symptoms being periodic hypersomnolence and excessive eating accompanied by behavioral changes. The dominance of the behavioral and psychological symptoms may obscure the diagnosis. In this article the diagnostic process and the psychiatric symptomatology of two adolescent male patients with kleine-levin syndrome is discussed. ( info) |
| OBJECTIVE: This study evaluated the possible pathologic relation between kleine-levin syndrome (KLS) and mood disorders. BACKGROUND: A 28-year-old man with a remote history of KLS had the sudden onset of a manic episode with psychotic features after the end of hypersomnolence. METHOD: The patient received an extensive laboratory examination, including single photon emission computed tomography and magnetic resonance imaging. RESULTS: Single photon emission computed tomography showed decreased tracer perfusion in the basal ganglion, hypothalamus, and right frontotemporal region. magnetic resonance imaging revealed a cystic lesion in the pineal region. CONCLUSIONS: Hypothalamic dysfunction has been described in KLS and mood disorders, but pineal gland dysfunction has been mentioned only rarely. The clinical and neuroimaging findings suggest the need for further study of KLS. ( info) |
4/23. 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. ( info) |
5/23. 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. ( info) |
| 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. ( info) |
| 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. ( info) |
8/23. 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. ( info) |
9/23. Familial kleine-levin syndrome: two siblings with unusually long hypersomnic spells. kleine-levin syndrome is a rare, sporadic disorder, with discrete spells of hypersomnolence occurring during adolescence, variously accompanied by megaphagia, behavioral changes, psychosis, and mild autonomic symptoms. Familial cases have not previously been reported. We describe 2 siblings who shared uncharacteristically prolonged episodes of hypersomnolence, and the HLA-DR2 haplotype. In one patient, levels of cerebrospinal fluid orexin (hypocretin) during an attack were normal. The presence of an increased sleep drive, despite the occurrence of large amounts of ostensibly restorative sleep, suggests the possible existence of a disorder of sleep satiety. ( info) |
| kleine-levin syndrome is characterized by hypersomnolence, hyperphagia and sexual disinhibition. The article reported a case of 10-year-old boy with a two-week history of altered sensorium, irrelevant talks, markedly increasing appetite and tendency to sleep most of the times. Immediately preceding to it the child had been an episode of enteric fever confirmed by the serological tests. ( info) |