Kleine-Levin syndrome in 120 patients: Differential diagnosis and long episodes
Objective Kleine–Levin syndrome is a rare disease characterized by recurrent episodes of hypersomnia with behavioral and cognitive disturbances. We aimed at describing the diagnosis procedure, risk factors, and severe forms. Methods In consecutive patients referred for suspected Kleine–Levin syndrom...
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Published in: | Annals of neurology Vol. 77; no. 3; pp. 529 - 540 |
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Main Authors: | , , , , , , , |
Format: | Journal Article |
Language: | English |
Published: |
United States
Blackwell Publishing Ltd
01-03-2015
Wiley Subscription Services, Inc Wiley |
Subjects: | |
Online Access: | Get full text |
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Summary: | Objective
Kleine–Levin syndrome is a rare disease characterized by recurrent episodes of hypersomnia with behavioral and cognitive disturbances. We aimed at describing the diagnosis procedure, risk factors, and severe forms.
Methods
In consecutive patients referred for suspected Kleine–Levin syndrome, we detailed differential diagnoses, and atypical and secondary cases, compared typical patients with healthy subjects, and examined the characteristics of patients with prolonged (>30 days) episodes.
Results
Among 166 referred patients, 120 had typical primary Kleine–Levin syndrome (syndrome secondary to brain diseases; n = 4, atypical syndrome, n = 7; differential diagnoses that were mostly psychiatric, n = 29; incomplete information, n = 6). The prevalence in France was 1.8 per million. The patients were often male (64%) and had more frequent birth and developmental abnormalities (45%) than controls (despite normal karyotypes), and most (80%) had teenage onset, with no difference between patients with prolonged (n = 34) and short (n = 85) episodes. In patients with prolonged episodes, the durations of the first episode (32 ± 33 vs 11 ± 6 days) and subsequent episodes were longer (mean episode duration = 23 ± 19 vs 10 ± 3 days) and the disease course tended to be longer (9 ± 6 vs 6 ± 4 years). During episodes, patients with prolonged episodes had shorter sleep time, higher levels of anxiety, increased agitation, and more feelings of disembodiment and amnesia. Between episodes, they were more tired, needed more naps, fell asleep more rapidly, and had higher anxiety/depression scores.
Interpretation
Mental disorders are frequent differential diagnoses of Kleine–Levin syndrome. One‐third of patients have prolonged (>1 month) episodes with more frequent immediate and long‐term consequences of the disease, prompting therapeutic trials. Ann Neurol 2015. Ann Neurol 2015;77:529–540 |
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Bibliography: | istex:B4AF73345C41656DD23981AFF82D1D6C50360BCC ArticleID:ANA24350 ark:/67375/WNG-7WCSTTNZ-7 ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 |
ISSN: | 0364-5134 1531-8249 |
DOI: | 10.1002/ana.24350 |