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Neurology 2002;59:1739-1745
© 2002 American Academy of Neurology

Kleine-Levin syndrome

An autoimmune hypothesis based on clinical and genetic analyses Y. Dauvilliers, MD, G. Mayer, MD, M. Lecendreux, MD, E. Neidhart, BSc, R. Peraita-Adrados, MD, K. Sonka, MD, M. Billiard, MD and M. Tafti, PhD

From Neurologie B (Drs. Dauvilliers and Billiard), Hôpital Gui-de-Chauliac, Montpellier, France; Hephata Klinik (Dr. Mayer), Schwalmstadt-Treysa, Germany; Service de Psychopathologie de l’Enfant et de l’Adolescent (Dr. Lecendreux), Hopital Robert Debré, Paris, France; Unité de Biochimie et Neurophysiologie Clinique (Drs. Dauvilliers and Tafti, and E. Neidhart), Département de Psychiatrie, Université de Genève, Switzerland; Unidad de Sueno (Dr. Peraita-Adrados), Hospital Universitario Gregorio Maranon, Madrid, Spain; and Department of Neurology (Dr. Sonka), Charles University, Prague, Czech Republic.

Address correspondence and reprint requests to Dr. Mehdi Tafti, HUG, Belle-Idée, Biochemistry and Genetics, Chemin du Petit-Bel-Air, 2, CH-1225 Chêne-Bourg, Switzerland; e-mail: Mehdi.Tafti{at}medecine.unige.ch

Background: Kleine-Levin syndrome (KLS) is a rare disorder of unknown etiology. Pathophysiologic hypotheses include a hypothalamic dysfunction and abnormalities in the central serotonin and dopamine metabolism. Several clinical symptoms also suggest an underlying autoimmune process.

Objective: To systematically investigate patients with KLS with reference to the available hypotheses.

Methods: The authors collected clinical, polysomnographic, CSF, CT, and MRI records and analyzed gene polymorphisms of HLA-DQB1, tryptophan hydroxylase (TpH), and catechol-O-methyltransferase (COMT) in 30 unrelated patients with KLS and their families. The genotype data were contrasted with data from a normal control population.

Results: Only human leukocyte antigen (HLA)-DQB1*0201 allele frequency was significantly increased in patients with KLS. Three patients with KLS but none of the control subjects were DQB1*0201 homozygous. Two affected subjects from the same family were DQB1*0201 homozygous. In 17 DQB1*0201 heterozygous parents, 11 (64.7%) had transmitted this allele, suggesting a preferential transmission.

Conclusion: These findings, together with the young age at onset, the recurrence of symptoms, and the frequent infectious precipitating factors, suggest an autoimmune etiology for Kleine-Levin syndrome.




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