Oussama Habra1, Raphaël Heinzer1, Jose Haba-Rubio1, Andrea O Rossetti1,2. 1. Center for Investigation and Research in Sleep, University Hospital and Faculty of Biology and Medicine, Lausanne, Switzerland. 2. Department of Clinical Neurosciences, University Hospital and Faculty of Biology and Medicine, Lausanne, Switzerland.
Abstract
STUDY OBJECTIVE: Kleine-Levin syndrome (KLS) is a rare disease of unknown etiology, the diagnosis of which can be challenging. We aimed to estimate KLS prevalence in French-speaking Switzerland, and assess differences with mimicking conditions. METHODS: In this cross-sectional study, KLS patients were identified through a population-based approach, including at our hospital and contacting all sleep-certified facilities and neurologists in French-speaking Switzerland. Furthermore, we identified patients referred to our center for suspected KLS that received other diagnoses. Relevant clinical data of these two groups was compared. RESULTS: We identified 7 patients with diagnosed KLS (6 since 2009), leading to a prevalence estimation of 3.19 per million (95% confidence interval: 1.55-6.59). Median age at diagnosis was 17 years (range: 12-19), 71.4% of them were men, and mean diagnosis delay after the first episode was 20.1 ± 10.9 months. We identified 9 mimic patients referred to our center; they differed from KLS patients by their higher age at disease onset (median: 15 [range: 12-16] vs. 19 [range: 16-64] years; p < 0.001), suspected KLS as referral reason (more frequent in mimics, p = 0.003), and the presence of precipitating factors (more frequent in KLS, p = 0.011). Among the mimic patients, 77% (versus 28% in KLS) had a psychiatric diagnosis. CONCLUSIONS: This study suggests a relatively higher KLS prevalence than previously reported. As compared to KLS, mimic patients have higher age at symptom onset, are more often initially referred for KLS suspicion, and have a higher prevalence of psychiatric disorders.
STUDY OBJECTIVE: Kleine-Levin syndrome (KLS) is a rare disease of unknown etiology, the diagnosis of which can be challenging. We aimed to estimate KLS prevalence in French-speaking Switzerland, and assess differences with mimicking conditions. METHODS: In this cross-sectional study, KLS patients were identified through a population-based approach, including at our hospital and contacting all sleep-certified facilities and neurologists in French-speaking Switzerland. Furthermore, we identified patients referred to our center for suspected KLS that received other diagnoses. Relevant clinical data of these two groups was compared. RESULTS: We identified 7 patients with diagnosed KLS (6 since 2009), leading to a prevalence estimation of 3.19 per million (95% confidence interval: 1.55-6.59). Median age at diagnosis was 17 years (range: 12-19), 71.4% of them were men, and mean diagnosis delay after the first episode was 20.1 ± 10.9 months. We identified 9 mimic patients referred to our center; they differed from KLS patients by their higher age at disease onset (median: 15 [range: 12-16] vs. 19 [range: 16-64] years; p < 0.001), suspected KLS as referral reason (more frequent in mimics, p = 0.003), and the presence of precipitating factors (more frequent in KLS, p = 0.011). Among the mimic patients, 77% (versus 28% in KLS) had a psychiatric diagnosis. CONCLUSIONS: This study suggests a relatively higher KLS prevalence than previously reported. As compared to KLS, mimic patients have higher age at symptom onset, are more often initially referred for KLS suspicion, and have a higher prevalence of psychiatric disorders.
Authors: José Haba-Rubio; John O Prior; Eric Guedj; Mehdi Tafti; Raphael Heinzer; Andrea O Rossetti Journal: Neurology Date: 2012-10-17 Impact factor: 9.910
Authors: Aditya Ambati; Ryan Hillary; Smaranda Leu-Semenescu; Hanna M Ollila; Ling Lin; Emmanuel H During; Neal Farber; Thomas J Rico; Juliette Faraco; Eileen Leary; Andrea N Goldstein-Piekarski; Yu-Shu Huang; Fang Han; Yakov Sivan; Michel Lecendreux; Pauline Dodet; Makoto Honda; Natan Gadoth; Sona Nevsimalova; Fabio Pizza; Takashi Kanbayashi; Rosa Peraita-Adrados; Guy D Leschziner; Rosa Hasan; Francesca Canellas; Kazuhiko Kume; Makrina Daniilidou; Patrice Bourgin; David Rye; José L Vicario; Birgit Hogl; Seung Chul Hong; Guiseppe Plazzi; Geert Mayer; Anne Marie Landtblom; Yves Dauvilliers; Isabelle Arnulf; Emmanuel Jean-Marie Mignot Journal: Proc Natl Acad Sci U S A Date: 2021-03-23 Impact factor: 11.205