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Published in: Sleep and Breathing 3/2018

Open Access 01-09-2018 | Sleep Breathing Physiology and Disorders • Review

Kleine-Levin syndrome: clues to aetiology

Authors: Saad Mohammed AlShareef, Richard Mark Smith, Ahmed Salem BaHammam

Published in: Sleep and Breathing | Issue 3/2018

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Abstract

Kleine-Levin syndrome (KLS) is the commonest recurrent sleep disorder, with a prevalence of 1–2 per million population. Clear diagnostic criteria are now defined, but effective treatment remains elusive. The significant body of published literature allows consideration of possible aetiological mechanisms, an understanding of which could guide the development of therapeutic strategies. Functional imaging studies have been inconclusive; although diencephalic abnormalities are a common finding, no consistent pattern has emerged, and these studies have not revealed the mechanism(s) underlying the development of the abnormalities detected. An autoimmune aetiology is consistent with the available data. In this review, we argue that, in order to further our understanding of KLS, there needs to be a co-ordinated international effort to standardise approaches to functional imaging studies, genetic analyses that specifically address the possibility of an autoimmune aetiology, and clinical trials of immunosuppressive therapies.
Literature
1.
go back to reference American Academy of Sleep Medicine. International Classification of Sleep Disorders - Third Edition (ICSD-3). (2014) Darien (IL): American Academy of Sleep Medicine American Academy of Sleep Medicine. International Classification of Sleep Disorders - Third Edition (ICSD-3). (2014) Darien (IL): American Academy of Sleep Medicine
24.
go back to reference Huang YS, Lin YH, Guilleminault C (2009) Polysomnography in Kleine-Levin syndrome. Neurology 70:795–801CrossRef Huang YS, Lin YH, Guilleminault C (2009) Polysomnography in Kleine-Levin syndrome. Neurology 70:795–801CrossRef
35.
go back to reference Chesson AL Jr, Levine SN, Kong LS, Lee SC (1991) Neuroendocrine evaluation in Kleine-Levin syndrome: evidence of reduced dopaminergic tone during periods of hypersomnolence. Sleep 14:226–232PubMed Chesson AL Jr, Levine SN, Kong LS, Lee SC (1991) Neuroendocrine evaluation in Kleine-Levin syndrome: evidence of reduced dopaminergic tone during periods of hypersomnolence. Sleep 14:226–232PubMed
49.
go back to reference ML L, Liu HC, Chen CH, Sung SM (2000) Kleine–Levin syndrome and psychosis: observation from an unusual case. Neuropsychiatry Neuropsychol Behav Neurol 13:140–142 ML L, Liu HC, Chen CH, Sung SM (2000) Kleine–Levin syndrome and psychosis: observation from an unusual case. Neuropsychiatry Neuropsychol Behav Neurol 13:140–142
52.
go back to reference Vigren P, Engstrom M, Landtblom AM (2014) SPECT in the Kleine-Levin syndrome, a possible diagnostic and prognostic aid? Front Neurol 5:1664–2295CrossRef Vigren P, Engstrom M, Landtblom AM (2014) SPECT in the Kleine-Levin syndrome, a possible diagnostic and prognostic aid? Front Neurol 5:1664–2295CrossRef
57.
go back to reference Portilla P, Durand E, Chalvon A, Habert M, Navelet Y, Prigent A, Landrieu P (2002) SPECT-identified hypoperfusion of the left temporomesial structures in a Kleine–Levin syndrome. Rev Neurol (Paris) 158(5 Pt 1):593–595 Portilla P, Durand E, Chalvon A, Habert M, Navelet Y, Prigent A, Landrieu P (2002) SPECT-identified hypoperfusion of the left temporomesial structures in a Kleine–Levin syndrome. Rev Neurol (Paris) 158(5 Pt 1):593–595
59.
go back to reference Engström M, Hallböök T, Szakacs A, Karlsson T, Landtblom AM (2014) Functional magnetic resonance imaging in narcolepsy and the Kleine–Levin syndrome. Front Neurol 5(105):42PubMedPubMedCentral Engström M, Hallböök T, Szakacs A, Karlsson T, Landtblom AM (2014) Functional magnetic resonance imaging in narcolepsy and the Kleine–Levin syndrome. Front Neurol 5(105):42PubMedPubMedCentral
65.
go back to reference Billings ME, Watson NF, Keogh BP (2011) Dynamic fMRI changes in Kleine-Levin syndrome. Sleep Med 12:531–532CrossRef Billings ME, Watson NF, Keogh BP (2011) Dynamic fMRI changes in Kleine-Levin syndrome. Sleep Med 12:531–532CrossRef
72.
go back to reference Merriam AE (1986) Kleine-Levin syndrome following acute viral encephalitis. Biol psychiatry (1993) a variant of the Kleine-Levin syndrome precipitated by both Epstein-Barr and varicella-zoster virus infections. Biol Psychiatry 33:388–390 Merriam AE (1986) Kleine-Levin syndrome following acute viral encephalitis. Biol psychiatry (1993) a variant of the Kleine-Levin syndrome precipitated by both Epstein-Barr and varicella-zoster virus infections. Biol Psychiatry 33:388–390
74.
go back to reference Silber MH (2016) Autoimmune sleep disorders handbook of clinical neurology, Vol. 133 (3rd series) pp 317-25 Autoimmune Neurology S.J. Pittock and A. Vincent, Editors Elsevier B.V Silber MH (2016) Autoimmune sleep disorders handbook of clinical neurology, Vol. 133 (3rd series) pp 317-25 Autoimmune Neurology S.J. Pittock and A. Vincent, Editors Elsevier B.V
96.
go back to reference Goldberg MA (1983) The treatment of Kleine-Levin syndrome with lithium. Canadian J Psychiatry 28(6):491–493CrossRef Goldberg MA (1983) The treatment of Kleine-Levin syndrome with lithium. Canadian J Psychiatry 28(6):491–493CrossRef
97.
go back to reference Mendhekar DN, Jiloha RC, Gupta D (2001) Kleine-levin syndrome: a report of two cases. Indian J Psychiatry 43(3):276–278PubMedPubMedCentral Mendhekar DN, Jiloha RC, Gupta D (2001) Kleine-levin syndrome: a report of two cases. Indian J Psychiatry 43(3):276–278PubMedPubMedCentral
107.
go back to reference de Oliveira MM, Conti C, Prado GF (2016) Pharmacological treatment for Kleine-Levin syndrome. Cochrane Database Syst Rev (5):Cd006685 de Oliveira MM, Conti C, Prado GF (2016) Pharmacological treatment for Kleine-Levin syndrome. Cochrane Database Syst Rev (5):Cd006685
Metadata
Title
Kleine-Levin syndrome: clues to aetiology
Authors
Saad Mohammed AlShareef
Richard Mark Smith
Ahmed Salem BaHammam
Publication date
01-09-2018
Publisher
Springer International Publishing
Published in
Sleep and Breathing / Issue 3/2018
Print ISSN: 1520-9512
Electronic ISSN: 1522-1709
DOI
https://doi.org/10.1007/s11325-017-1617-z

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