| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
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.
This article has been cited by other articles:
|
|
 |
|
  Y.-S. Huang, Y.-H. Lin, and C. Guilleminault Polysomnography in Kleine-Levin syndrome Neurology, March 4, 2008; 70(10): 795 - 801. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 O. Walusinski Observation of a nervous disease attended by disturbed sleep, at times lethargic and at times convulsive. Edme Chauvot de Beauchene (1786) J. Neurol. Neurosurg. Psychiatry, September 1, 2007; 78(9): 975 - 976. [Full Text] [PDF]
|
|
|
|
 |
|
 T. J. Young and M. H. Silber Hypersomnias of central origin. Chest, September 1, 2006; 130(3): 913 - 920. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 I. Arnulf, J. M. Zeitzer, J. File, N. Farber, and E. Mignot Kleine-Levin syndrome: a systematic review of 186 cases in the literature Brain, December 1, 2005; 128(12): 2763 - 2776. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Y Dauvilliers, C. Baumann, B Carlander, M Bischof, T Blatter, M Lecendreux, F Maly, A Besset, J Touchon, M Billiard, et al. CSF hypocretin-1 levels in narcolepsy, Kleine-Levin syndrome, and other hypersomnias and neurological conditions J. Neurol. Neurosurg. Psychiatry, December 1, 2003; 74(12): 1667 - 1673. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
