Kleine-Levin Syndrome (Syndrome, Kleine-Levin)

A rare condition characterized by recurrent hypersomnias associated with hyperphagia, occurring primarily in males in the second to third decade of life. Clinical features include mental confusion, excessive sleep requirements (approximately 18 hours per day), restlessness, and in some cases hallucinations. Episodes have a duration of days to weeks, and may recur several times per year. This condition may resolve spontaneously over several years. (From Adams, et al., Principles of Neurology, 6th ed, p569)

Also Known As:

Networked: 87 relevant articles (5 outcomes, 2 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Epilepsy (Aura)
2.	Disorders of Excessive Somnolence (Hypersomnia)
3.	Kleine-Levin Syndrome (Syndrome, Kleine-Levin)
4.	Narcolepsy
5.	Multiple Sclerosis

Experts

1.	Arnulf, Isabelle: 4 articles (01/2021 - 08/2015)
2.	Dauvilliers, Yves: 4 articles (01/2016 - 12/2006)
3.	Groos, Elisabeth: 3 articles (01/2021 - 11/2015)
4.	Conti, Cristiane: 3 articles (05/2016 - 04/2009)
5.	Dhossche, Dirk M: 3 articles (04/2014 - 11/2010)
6.	Kanbayashi, Takashi: 2 articles (01/2022 - 01/2018)
7.	Trotti, Lynn Marie: 2 articles (01/2021 - 04/2014)
8.	Watson, Nathaniel F: 2 articles (01/2021 - 11/2013)
9.	Kodaira, Minori: 2 articles (01/2018 - 01/2012)
10.	Leu-Semenescu, Smaranda: 2 articles (01/2018 - 11/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Kleine-Levin Syndrome:

1.	LithiumIBA 07/01/2002 - "Efficacy of lithium treatment in Kleine-Levin syndrome." 10/01/1983 - "A case of Kleine-Levin Syndrome without megaphagia is described in an adolescent male who responded well to lithium treatment. " 01/01/2022 - "Two cases of Kleine-Levin syndrome with good responses to lithium treatment at low serum levels." 01/01/2021 - " We suggest that clinicians use lithium for the treatment of Kleine-Levin syndrome in adults. " 07/01/2017 - "Successful Long-term Management of a Child with Kleine-Levin Syndrome with Low-dose Lithium."
2.	Carbamazepine (Tegretol)FDA LinkGeneric 07/01/2009 - "A case of Kleine-Levin syndrome with a complete and sustained response to carbamazepine." 07/01/1999 - "Carbamazepine for Kleine-Levin syndrome." 07/01/1997 - "MENSTRUATION RELATED PERIODIC HYPERSOMNIA SUCCESSFUL OUTCOME WITH CARBAMAZEPINE (A Case Report)." 07/05/1986 - "[A case of Kleine-Levin syndrome stabilized for over 1 year with carbamazepine]." 07/01/2009 - "We describe a patient with Kleine-Levin syndrome who was initially misdiagnosed as having epilepsy and who achieved complete remission on carbamazepine treatment. "
3.	Lithium Carbonate (Eskalith)FDA LinkGeneric 10/01/1983 - "Recent reports, including the present one, strongly suggest that Lithium Carbonate may be the treatment of choice for the Kleine-Levin Syndrome." 04/01/2014 - "Kleine-Levin syndrome may present with hyperorality, and our patient responded well to lithium carbonate therapy." 01/01/1988 - "On the other hand, the prevention of the hypersomniac episodes of the Kleine Levin syndrome with lithium carbonate has been successful in several well-documented cases as well as the prevention of the hypersomniac episodes of the menstruation related hypersomnia with ovulatory inhibitors. " 09/01/1987 - "In addition to its predominant use for the prophylaxis of recurrent mania and depression, lithium carbonate has been proposed and tried in the prophylactic treatment of abnormally prolonged sleep episodes featuring the Kleine-Levin syndrome."
4.	Gabapentin (Neurontin)FDA LinkGeneric 01/01/2009 - "We describe a 17-year-old girl with Kleine-Levin syndrome (KLS), in which gabapentin was effective for the prevention of attacks. " 01/01/2009 - "Gabapentin for Kleine-Levin syndrome."
5.	Antidepressive Agents (Antidepressants)IBA 01/01/2013 - "However, no single drug therapy has been consistently successful, despite various psychotropic agents, including lithium, anticonvulsants and antidepressants, having been systematically tried (Arnulf et al., "Kleine-Levin syndrome: a systematic study of 108 patients." Ann Neurol 2008;63:482-492). "
6.	Anticonvulsants (Antiepileptic Drugs)IBA 01/01/2013 - "However, no single drug therapy has been consistently successful, despite various psychotropic agents, including lithium, anticonvulsants and antidepressants, having been systematically tried (Arnulf et al., "Kleine-Levin syndrome: a systematic study of 108 patients." Ann Neurol 2008;63:482-492). "
7.	OrexinsIBA 04/01/2016 - "Cerebrospinal Fluid Orexin A Levels and Autonomic Function in Kleine-Levin Syndrome." 12/01/2006 - "Kleine-Levin syndrome in a 14-year-old girl: CSF hypocretin-1 measurements." 01/01/2018 - "Fluctuating CSF hypocretin-1 levels in mild brain trauma-induced Kleine-Levin syndrome." 12/01/2006 - "CSF hypocretin-1 measurements were performed during a period of hypersomnia and during an asymptomatic interval in a 14-year-old girl affected with severe Kleine-Levin syndrome. " 08/01/2011 - "A second part of the review considers the main current issues on recurrent hypersomnia: the predisposing factors, including a window on family cases; the pathophysiology based on clinical patterns, neuroimaging data, neuropathological examinations and cerebrospinal fluid (CSF) hypocretin-1 measurements; the issues of recurrence and of a possible disruption of the circadian timing system; the relationships between recurrent hypersomnia and mood disorders; and a note on the atypical Kleine-Levin syndrome. "
8.	Biomarkers (Surrogate Marker)IBA 01/01/2022 - "Proteomic biomarkers of Kleine-Levin syndrome." 01/01/2016 - "Time to find a biomarker in Kleine-Levin Syndrome." 01/01/2015 - "To measure CSF biomarkers of hypothalamic dysfunction in patients with typical Kleine-Levin syndrome (KLS) during symptomatic and asymptomatic periods. " 01/01/2015 - "Preliminary results on CSF biomarkers for hypothalamic dysfunction in Kleine-Levin syndrome." 01/01/2014 - "In the Kleine Levin syndrome, fMRI has identified hyperactivation of the thalamus as a potential biomarker that could be used in the diagnostic procedure. "
9.	Valproic Acid (Depakote)FDA LinkGeneric 07/01/2015 - "Successful Use of Valproate in Kleine-Levin Syndrome: A Case Report and Review of Cases Reported from India." 01/01/2013 - "[Invasive Kleine-Levin syndrome responding to valproate]." 03/01/2009 - "An adult onset patient with Kleine-Levin syndrome responding to valproate." 07/01/1997 - "Valproic acid for Kleine-Levin syndrome."
10.	Modafinil (Provigil)FDA Link 01/01/2013 - "Modafinil has been reported to show an effect in shortening the duration of symptomatic periods, but does not affect the recurrence rate (Huang et al., "Kleine-Levin syndrome: current status." Med Clin N Am 2010;94:557-562). " 01/01/2011 - "Kleine-Levin syndrome and response to modafinil in a young woman." 06/01/2010 - "Modafinil-associated vivid visual hallucination in a patient with Kleine-Levin syndrome: case report." 12/01/2001 - "Several unpublished cases are presented: one case of pseudohypersomnia due to a bilateral thalamic infarct and corrected by modafinil, four probable late-onset autosomal recessive cerebellar ataxias without sleep pattern anomalies, six cases of fronto-temporal dementia with strong reduction in total sleep time and REMS percentage on the first polysomnographic night, one case of periodic hypersomnia associated with a Rathke's cleft cyst and four cases of suspected symptomatic narcolepsy with a DR16-DQ5 haplotype, three of which were post-traumatic without MRI anomalies, and one associated with multiple sclerosis exhibiting pontine hyper signals on MRI."

Therapies and Procedures

1.	Therapeutics 04/15/2009 - "Some medications have been reported to provide some benefit for the treatment of Kleine-Levin Syndrome patients, but because of the rarity of the condition no long-term follow-up therapies have yet been described. " 04/01/2014 - "Kleine-Levin syndrome may present with hyperorality, and our patient responded well to lithium carbonate therapy." 11/10/2015 - "Lithium therapy in Kleine-Levin syndrome: An open-label, controlled study in 130 patients." 11/10/2015 - "To compare the benefits and risks of lithium therapy vs abstention/other treatments in Kleine-Levin syndrome (KLS). " 09/01/1985 - "Kleine-Levin syndrome: normal CSF monoamines and response to lithium therapy."
2.	Drug Therapy (Chemotherapy) 01/01/2013 - "Effects of various drug therapies on Kleine-Levin syndrome: a case report." 01/01/2019 - "Other possible indications, with limited published evidence, are the acute attacks in Kleine-Levin syndrome, behavioral symptoms of X-fragile syndrome, and the management of clozapine- or chemotherapy- induced neutropenia. " 01/01/2013 - "However, no single drug therapy has been consistently successful, despite various psychotropic agents, including lithium, anticonvulsants and antidepressants, having been systematically tried (Arnulf et al., "Kleine-Levin syndrome: a systematic study of 108 patients." Ann Neurol 2008;63:482-492). "
3.	General Anesthesia 12/01/2021 - "Kleine-Levin syndrome and general anesthesia: A case report." 09/01/2022 - "Perioperative Considerations in a Patient with Kleine-Levin Syndrome Undergoing a Neurosurgical Procedure under General Anesthesia."
4.	Low-Level Light Therapy (LLLT) 10/01/2021 - "Treatment of Kleine-Levin Syndrome With Intranasal Photobiomodulation and Methylene Blue."
5.	Neurosurgical Procedures 09/01/2022 - "Perioperative Considerations in a Patient with Kleine-Levin Syndrome Undergoing a Neurosurgical Procedure under General Anesthesia."