Brain Advance Access originally published online on October 17, 2005
Brain 2005 128(12):2763-2776; doi:10.1093/brain/awh620
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Review Article |
Kleine–Levin syndrome: a systematic review of 186 cases in the literature
1 Stanford University Center for Narcolepsy, 2 Howard Hughes Medical Institute, Palo Alto, CA, 3 Kleine–Levin Syndrome Foundation, Boston, MA, USA and 4 Fédération des Pathologies du Sommeil, Hôpital Pitié-Salpêtrière, Paris, France
Correspondence to: I. Arnulf, Fédération des pathologie du Sommeil, Hôpital Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital, 75013 Paris, France E-mail: isabelle.arnulf{at}psl.ap-hop-paris.fr
Kleine–Levin syndrome (KLS) is a rare disorder with symptoms that include periodic hypersomnia, cognitive and behavioural disturbances. Large series of patients are lacking. In order to report on various KLS symptoms, identify risk factors and analyse treatment response, we performed a systematic review of 195 articles, written in English and non-English languages, which are available on Medline dating from 1962 to 2004. Doubtful or duplicate cases, case series without individual details and reviews (n = 56 articles) were excluded. In addition, the details of 186 patients from 139 articles were compiled. Primary KLS cases (n = 168) were found mostly in men (68%) and occurred sporadically worldwide. The median age of onset was 15 years (range 4–82 years, 81% during the second decade) and the syndrome lasted 8 years, with seven episodes of 10 days, recurring every 3.5 months (median values) with the disease lasting longer in women and in patients with less frequent episodes during the first year. It was precipitated most frequently by infections (38.2%), head trauma (9%), or alcohol consumption (5.4%). Common symptoms were hypersomnia (100%), cognitive changes (96%, including a specific feeling of derealization), eating disturbances (80%), hypersexuality (43%), compulsions (29%), and depressed mood (48%). In 75 treated patients (213 trials), somnolence decreased using stimulants (mainly amphetamines) in 40% of cases, while neuroleptics and antidepressants were of poor benefit. Only lithium (but not carbamazepine or other antiepileptics) had a higher reported response rate (41%) for stopping relapses when compared to medical abstention (19%). Secondary KLS (n = 18) patients were older and had more frequent and longer episodes, but had clinical symptoms and treatment responses similar to primary cases. In conclusion, KLS is a unique disease which may be more severe in female and secondary cases.
Key Words: hypersexuality; hypersomnia; Kleine–Levin syndrome; megaphagia; periodic; recurrent
Abbreviations: KLS = Kleine–Levin syndrome; REM sleep = rapid eye movement sleep
Received April 9, 2005. Revised July 14, 2005. Accepted July 19, 2005.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  G Stores Clinical diagnosis and misdiagnosis of sleep disorders J. Neurol. Neurosurg. Psychiatry, December 1, 2007; 78(12): 1293 - 1297. [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]
|
|