Increased cerebrospinal fluid 5‐hydroxytryptamine and 5‐hydroxyindoleacetic acid in Kleine‐Levin syndrome

Koerber, Rodney K.; Torkelson, Richard; Haven, Guy T.; Donaldson, John; Cohen, Samuel M.; Case, Mary Anne

doi:10.1212/wnl.34.12.1597

Cited by 68 publications

(23 citation statements)

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“…The superior effect of amphetamines on KLS may be related to their impact on serotonergic mechanisms. Although the data were not consistent in support of this hypothesis, Koerber et al (1984) reported that serotonin and serotonin metabolite levels in the cerebrospinal fluid of a KLS patient were higher than expected. Studies on the relationship between sleep and serotonergic activity indicate that agonistic activity on 5-HT1A, 5-HT1B, 5-HT2A, 5-HT2C, and 5-HT3 receptors provides wakefulness (Monti and Jantos 2008;Monti 2011).…”

Section: Discussionmentioning

confidence: 75%

Treatment of Kleine–Levin Syndrome with Aripiprazole

Savaş

Akça

Bi̇lgi̇ç

2015

Journal of Child and Adolescent Psychopharmacology

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K leine-Levin syndrome (KLS) is a rare and disabling disorder characterized by recurrent episodes of hypersomnia as well as cognitive and behavioral abnormalities such as compulsive hyperphagia and hypersexual behavior. These episodes usually last a few days to weeks; however, the recurring nature of the syndrome can disrupt the individual's social, professional, and family life. Furthermore, irritability/hostility, social disinhibition, obscene language, depersonalization/derealization, and depressive and/or manic symptoms can co-occur during the episodes (Arnulf et al. 2005). Although the etiology of KLS is not well known, various drugs, including stimulants, lithium, antiepileptics, antipsychotics, and antidepressants have been used in the treatment of the disorder (Oliveira et al. 2013). However, there have been no reports on the effectiveness of aripiprazole on KLS. Herein, we present an adolescent with KLS who displayed complete remission after aripiprazole therapy. Case ReportThe patient was a 15-year-old girl who had experienced four recurrent episodes -lasting for * 10 days -of severe sleepiness within a 4 month span. During these episodes, she was sleeping most of the day. She could be awakened, but was confused and was unable to maintain concentration or recall recent events during the awakened times of the sleep episodes. Her family described her awake times as ''like in a dream.'' After the sleepy period, she had an overactive, less sleeping, and anxious period in which she showed erotomanic and referential preoccupations, derealization, compulsive behaviors (i.e., frequent hand washing), and hyperphagia. This episode ended after 7 days and was followed by an asymptomatic period lasting 1 week. Thereafter, another sleepy episode began, and episode durations were similar for all four episodes. During the asymptomatic phase, the patient described partial amnesia for the events that had occurred. She denied any hypersexual behavior during or after the episodes. She had been admitted to a child psychiatry clinic with these complaints and was administered sertraline (25 mg), haloperidol (1 mg), and imipramine at different times. However, no improvement was observed with these medications. She was seen by a neurologist who observed no epileptic activity or neurological pathology. She had normal electroencephalogram (EEG) and brain magnetic resonance imaging (MRI) reports. To support the KLS diagnosis, human leukocyte antigen (HLA) analyses (DQB1) were studied in a genetic laboratory, based on the literature indicating that this antigen group may be related to KLS (Huang et al. 2012). The DQB1*06 and DQB1*03 antigens were found to be positive.We had diagnosed this patient as having KLS based on the clinical findings and HLA analyses results, according to the International Classification of Sleep Disorders-II (American Academy of Sleep Medicine 2005). The patient's medications were terminated upon admission, and aripiprazole (5 mg/day) was started to target the erotomanic and referential preoccupations durin...

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Section: Discussionmentioning

confidence: 75%

Treatment of Kleine–Levin Syndrome with Aripiprazole

Savaş

Akça

Bi̇lgi̇ç

2015

Journal of Child and Adolescent Psychopharmacology

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“…Mixed evidence suggests hypothalamic dysfunction in KLS (Koerber et al 1984;Gadoth et al 1987;Fernábdez et al 1990;Chesson et al 1991;Malhotra et al 1997;Mayer et al 1998;Kas et al 2014). Evidence for thalamic involvement also exists; Tc-99m ethyl cysteinate dimer (ECD) single-photon emission CT analysis during symptomatic periods of KLS has demonstrated thalamic hypoperfusion (Huang et al 2005;Hong et al 2006;Huang et al 2012b;Kas et al 2014).…”

Section: Discussionmentioning

confidence: 99%

PANDAS and Comorbid Kleine–Levin Syndrome

Gerardi

Casadonte

Patel

et al. 2015

Journal of Child and Adolescent Psychopharmacology

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“…Excitatory monoamines are either increased 4 or within normal limits in the CSF of KLS patients, 5 explaining the limited effectiveness of stimulants, which increase CSF monoamines, in treating this syndrome. This lack of monoaminergic involvement suggests a pathogenesis rooted in naturally occurring excess GABAergic signaling.…”

Section: Discussionmentioning

confidence: 99%

Kleine-Levin Syndrome Treated With Clarithromycin

Rezvanian

Watson

2013

Journal of Clinical Sleep Medicine

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Kleine-Levin syndrome (KLS) is a rare sleep disorder characterized by periodic hypersomnia and various degrees of cognitive and behavioral disturbance, hyperphagia, and hypersexuality. Effective treatment is challenging. Stimulants marginally address sleepiness, but may increase irritability and do not improve cognitive and behavioral disturbances. Modafi nil may shorten the symptomatic period but not the recurrence rate. Lithium and carbamazepine are benefi cial in some cases, possibly related to similarities between KLS and affective disorders. Currently, no single medication is consistently successful in treating the syndrome. Here we report the shortterm effect of clarithromycin in a patient with KLS. C A S E R E P O R T SK leine-Levin Syndrome (KLS) is a rare sleep disorder of unknown cause characterized by repetitive, intermittent cycles of extreme sleepiness, and cognitive/behavioral disturbances including confusion, feelings of unreality, aggressiveness, and hypersexuality. Gamma-aminobutyric acid (GABA) is the predominant inhibitory neurotransmitter in the brain with well-known effects on sleep/wake cycle regulation. The GABA A receptor is the site where sleep-inducing drugs such as diazepam have their effects, and recent research suggests GABA A receptor antagonists may be useful in the treatment of hypersomnia.1 We sought to learn if clarithromycin, an antibiotic with GABA A receptor antagonistic properties, would be benefi cial in the treatment of KLS. REPORT OF CASEWe report a 23-year-old female with KLS beginning at 10 years of age. Her diagnosis was consistent with ICSD-2 criteria and based on recurrent symptoms of hypersomnia, hypersexuality, irritability, and child-like behavior with a dreamlike state.2 KLS episode triggers included alcohol, upper respiratory infections, and general anesthesia. She slept 14 to 18 h/night during hypersomnia episodes, which could last from weeks to months. She typically had headache, nausea, and vomiting at episode onset. MRI perfusion showed episode related reductions in thalamic perfusion.3 Episode length and duration were variable, but over time they had decreased in severity allowing performance of some of her daily life functions. Between episodes her sleep duration was approximately 9 h/night with normal mood and activity levels. She had no comorbid illness and took no regular medications. She had tried a number of medications for KLS, including prazosin, acetazolamide, methylphenidate, and liothyronine, all of which were discontinued due to either side effects or ineffectiveness. In the midst of a recent episode she had been started on clarithromycin 500 mg every 12 hours. She was on no other medications at that time. Within 24 h, her sleepiness

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Increased cerebrospinal fluid 5‐hydroxytryptamine and 5‐hydroxyindoleacetic acid in Kleine‐Levin syndrome

Cited by 68 publications

References 0 publications

Treatment of Kleine–Levin Syndrome with Aripiprazole

Treatment of Kleine–Levin Syndrome with Aripiprazole

PANDAS and Comorbid Kleine–Levin Syndrome

Kleine-Levin Syndrome Treated With Clarithromycin

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