A Case of Kleine-Levin Syndrome: Diagnostic and Therapeutic Challenge
,
Abstract
Kleine-Levin syndrome (KLS) is a rare sleep disorder mainly affecting teenage boys in which the main features are intermittent hypersomnolence, behavioral and cognitive disturbances, hyperphagia, and in some cases hyper sexuality. Etiology is unknown, and there is no specific clinical or imaging test for this syndrome even though the illness has well-defined clinical features. Also, there is no effective treatment for KLS. KLS is self-limited, so the prognosis for these patients is not so bad. This study presents our case report and comprehensive workout that led to diagnosis which is primarily clinical. Our patient is a 20-year-old man referred to our clinic because of sleeping problems. At the age of 14, he presented with complaints of the excessive duration of sleep, increased appetite, excessive daytime sleepiness, loss of interest in social activities during attendance of high school and hallucinations. The excessive diagnostic procedure does not find pathological. Kleine-Levin syndrome (KLS) is a rare sleep disorder of unknown etiology which diagnosis is clinical and diagnostic workup is mainly to exclude other similar conditions. There is no specific therapy, but the disease is self-limited and with good prognosis.
Hauri P, editor. American Academy of Sleep Medicine. The International Classification of Sleep Disorders—Revised Diagnostic and Coding Manual. Chicago, IL: American Academy of Sleep Medicine; 2005. p. 297.
Kleine W. Periodische schlafsucht. Monats Psychiatr Neurol. 1925;57:285–320.
Levin M. Periodic somnolence and morbid hunger: A new syndrome. Brain. 1936;59:494–504.
Levin M. Narcolepsy (Gelineau’s syndrome) and other varieties of morbid somnolence. Arch Neurol Psychiat. 1929;22:1172–200.
Critchley M. Periodic hypersomnia and megaphagia in adolescent males. Brain. 1962;85:627–56. [PubMed: 14023898]
Thorpy MJ. Chairman. International Classification of Sleep Disorders: Diagnostic and Coding Manual. Diagnostic Classification Steering Committee. American Sleep Disorders Association. 1990
Arnulf I, Zeitzer J, File J, Farber N, Mignot E. Kleine-Levin syndrome: A systematic review of 186 cases in the literature. Brain. 2005;128:2763–76. [PubMed: 16230322]
Gadoth N, Kesler A, Vainstein G, Peled R, Lavie P. Clinical and polysomnographic characteristics of 34 patients with Kleine-Levin syndrome. J Sleep Res. 2001;10:337–41. [PubMed: 11903864]
Huang YS, Arnulf I. The Kleine-Levin Syndrome. Sleep Med Clin. 2006;1:89–103.
Janicki S, Franco K, Zarko R. A case report of Kleine-Levin syndrome in an adolescent girl. Psychosomatics. 2001;42:350–2. [PubMed: 11496026]
Katz JD, Ropper AH. Familial Kleine-Levin syndrome: Two siblings with unusually long hypersomnic spells. Arch Neurol. 2002;59:1959–61. [PubMed: 12470186]
Huang YS, Guilleminault C, Kao PF, Liu FY. SPECT findings in the Kleine-Levin syndrome. Sleep. 2005;28:955–60. [PubMed: 16218078]
Merriam AE. Kleine-Levin syndrome following acute viral encephalitis. Biol Psychiatry. 1986;21:1301–4. [PubMed: 3463365]
Dauvilliers Y, Mayer G, Lecendreux M, Neidhart E, Peraita-Adrados R, Sonka K, et al. Kleine-Levin syndrome: An autoimmune hypothesis based on clinical and genetic analyses. Neurology. 2002;59:1739–45. [PubMed: 12473762]
Fenzi F, Simonati A, Crosato F, Ghersini L, Rizzuto N. Clinical features of Kleine–Levin syndrome with localized encephalitis. Neuropediatrics. 1993;24:292–5. [PubMed: 8309520]
Chesson A, Levine S, Kong LS, Lee S. Neuroendocrine evaluation in Kleine-Levin Syndrome: Evidence of reduced dopaminergic tone during periods of hypersomnolence. Sleep. 1991;14:226–32. [PubMed: 1654588]
Koerber RK, Torkelson R, Haven G, Donaldson J, Cohen SM, Case M. Increased cerebrospinal fluid 5-hydroxytryptamine and 5- hydroxyindoleacetic acid in Kleine-Levin syndrome. Neurology. 1984;34:1597–600. [PubMed: 6209595]
Prabhakaran N, Murthy GK, Mallya UL. A case of Kleine–Levin syndrome in India. Br J Psychiatry. 1970;117:517–9. [PubMed: 5480699]
Gallinek A. The Kleine–Levin syndrome. Dis Nerv Syst. 1967;28:448–51. [PubMed: 4951979]
Russell J, Grunstein R. Kleine–Levin syndrome: A case report. Aust N Z J Psychiatry. 1992;26:119–23. [PubMed: 1580874]
Sagar RS, Khandelwal SK, Gupta S. Interepisodic morbidity in Kleine–Levin syndrome. Br J Psychiatry. 1990;157:139–41. [PubMed: 2397348]
Masi G, Favilla L, Millepiedi S. The Kleine–Levin syndrome as a neuropsychiatric disorder: A case report. Psychiatry. 2000;63:93–100. [PubMed: 10855764]
Landtblom AM, Dige N, Schwerdt K, Safstrom P, Granerus G. Short-term memory dysfunction in Kleine–Levin syndrome. Acta Neurol Scand. 2003;108:363–7. [PubMed: 14616308]
Smolik P, Roth B. Kleine–Levin syndrome etiopathogenesis and treatment. Acta Univ Carol Med Monogr. 1988;128:5–94. [PubMed: 3078227]
Shukla G, Bajpai H, Mishra D. Kleine–Levin syndrome: A case-report from India. Br J Psychiatry. 1982;141:97–104. [PubMed: 6956423]
Poppe M, Friebel D, Reuner U, Todt H, Koch R, Heubner G. The Kleine– Levin syndrome—effects of treatment with lithium. Neuropediatrics. 2003;34:113–9. [PubMed: 12910433]
Reynolds CF, Black RS, Coble P, Holzer B, Kupfer DJ. Similarities in EEG sleep findings for Kleine–Levin syndrome and unipolar depression. Am J Psychiatry. 1980;137:116–8. [PubMed: 6928091]
Goldberg MA. The treatment of Kleine–Levin syndrome with lithium. Can J Psychiatry. 1983;28:491–3. [PubMed: 6416662]
Yassa R, Nair NP. The Kleine–Levine syndrome—a variant? J Clin Psychiatry. 1978;39:254–9. [PubMed: 273020]
Fresco R, Giudicelli S, Poinso Y, Tatossian A, Mouren P. Kleine–Levin syndrome—recurrent hypersomnia of male adolescents. Ann Med Psychol (Paris) 1971;1:625–68. [PubMed: 5559004]
Huang YS, Lin YH, Guilleminault C. Polysomnography in Kleine-Levin syndrome. Neurology. 2009;70:795–801. [PubMed: 18316691]
Papacostas SS. Photosensitivity during the hypersomnic phase in a patient with Kleine–Levin syndrome. J Child Neurol. 2003;18:432–3. [PubMed: 12886982]
Hong SB, Joo EY, Tae WS. Episodic diencephalic hypoperfusion in Kleine-Levin syndrome. Sleep. 2006;29:1091–3. [PubMed: 16944679]
Oliveira MM, Conti C, Saconato H, Fernandes do Prado G. Pharmacological treatment for Kleine-Levin Syndrome. Cochrane Database Syst Rev. 2009;2:CD006685. [PubMed: 19370648]
Mukaddes NM, Alyanak B, Kora ME, Polvan O. The psychiatric symptomatology in Kleine–Levin syndrome. Child Psychiatry Hum Dev. 1999;29:253–8. [PubMed: 10080967]
Arnulf I, Lin L, Gadoth N, File J, Lecendreux M, Franco P, et al. Kleine–Levin Syndrome: A Systematic Study of 108 Patients. Ann Neurol. 2008;63:482–92. [PubMed: 18438947]
Dauvilliers Y, Baumann CR, Carlander B, Bischof M, Blatter T, Lecendreux M, et al. CSF hypocretin-1 levels in narcolepsy, Kleine–Levin syndrome, and other hypersomnias and neurological conditions. J Neurol Neurosurg Psychiatry. 2003;74:1667–73. [PMCID: PMC1757412] [PubMed: 14638887]
| Files | ||
| Issue | Vol 56, No 1 (2018) | |
| Section | Case Report(s) | |
| Keywords | ||
| Hypersomnia Hyper sexuality Kleine-Levin syndrome Megaphagia Periodic | ||
| Rights and permissions | |
|
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
