Publication date: Jul 01, 2025
Kleine-Levin Syndrome (KLS) is a neurological disorder of unknown pathophysiology. It is characterized by relapsing-remitting episodes of hypersomnia, with cognitive symptoms and behavioral disturbances. The diagnosis relies on clinical criteria, which require further standardization. Our main objective was to better phenotype and systematically quantify the severity and impact of cognitive and behavioral symptoms, hypersomnolence and dysautonomia in a well-characterized population of KLS patients. Forty-three consecutive KLS patients diagnosed in a National Reference Center for Rare Hypersomnias underwent a standardized clinical and polysomnographic evaluation and completed validated questionnaires with their relatives. The Behavioral Dysexecutive Syndrome Inventory (BDSI) assessed their behavior during most episodes. The Idiopathic-Hypersomnia Severity Scale (IHSS) assessed different hypersomnolence components (inertia, sleepiness, prolonged sleep), and the Scale for Outcomes in Parkinson’s Disease-Autonomic (SCOPA-AUT) assessed dysautonomia. The latter two scales were completed twice to differentiate symptoms during and between episodes. During symptomatic periods, behavioral changes were observed in all BDSI domains, more than half of patients showing reduced activities and apathy, anticipation-organization-initiation difficulties, disinterest, irritability-aggression, perseveration-stereotypies, and sexual or eating disorders. IHSS scores were severe and higher during episodes (42. 6 +/- 5. 8 vs. 15. 6 +/- 9. 8, p
Semantics
| Type | Source | Name |
|---|---|---|
| disease | MESH | Syndrome |
| disease | MESH | Hypersomnolence |
| disease | MESH | Dysautonomia |
| disease | MESH | Kleine-Levin Syndrome |
| disease | MESH | neurological disorder |
| drug | DRUGBANK | Tropicamide |
| disease | MESH | sleepiness |
| disease | MESH | Parkinson’s Disease |
| disease | MESH | apathy |
| disease | MESH | eating disorders |
| disease | MESH | Primary Dysautonomias |