{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T23:33:54Z","timestamp":1789256034657,"version":"build-2803163510"},"reference-count":32,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2025,6,27]],"date-time":"2025-06-27T00:00:00Z","timestamp":1750982400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Kleine\u2010Levin Syndrome (KLS) is a neurological disorder of unknown pathophysiology. It is characterized by relapsing\u2013remitting 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\u2010characterized population of KLS patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Forty\u2010three 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\u2010Hypersomnia Severity Scale (IHSS) assessed different hypersomnolence components (inertia, sleepiness, prolonged sleep), and the Scale for Outcomes in Parkinson's Disease\u2010Autonomic (SCOPA\u2010AUT) assessed dysautonomia. The latter two scales were completed twice to differentiate symptoms during and between episodes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      During symptomatic periods, behavioral changes were observed in all BDSI domains, more than half of patients showing reduced activities and apathy, anticipation\u2013organization\u2013initiation difficulties, disinterest, irritability\u2013aggression, perseveration\u2013stereotypies, and sexual or eating disorders. IHSS scores were severe and higher during episodes (42.6\u2009\u00b1\u20095.8 vs. 15.6\u2009\u00b1\u20099.8,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.0001), and each item higher. The SCOPA\u2010AUT scores were higher during episodes (12.1\u2009\u00b1\u20098.5 vs. 8.5\u2009\u00b1\u20098.2,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.0001), particularly in the cardiovascular, pupillomotor, and thermoregulation domains.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Our findings suggest the relevance of the BDSI for the evaluation of dysexecutive behavioral symptoms in KLS. The IHSS would be useful for phenotyping hypersomnolence and the SCOPA\u2010AUT for assessing dysautonomia during episodes. Future studies could combine the BDSI with imaging and other biomarkers to explore the neuroanatomical correlates of this enigmatic disorder.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/ene.70259","type":"journal-article","created":{"date-parts":[[2025,6,27]],"date-time":"2025-06-27T09:40:06Z","timestamp":1751017206000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Systematic Assessment of Dysexecutive Syndrome, Hypersomnolence and Dysautonomia in Kleine\u2010Levin Syndrome"],"prefix":"10.1111","volume":"32","author":[{"given":"Lucie","family":"Barateau","sequence":"first","affiliation":[{"name":"Sleep\u2010Wake Disorders Unit, Department of Neurology Gui\u2010de\u2010Chauliac Hospital, CHU Montpellier  Montpellier France"},{"name":"National Reference Centre for Orphan Diseases, Narcolepsy, Idiopathic Hypersomnia, and Kleine\u2010Levin Syndrome  Montpellier France"},{"name":"Institute of Neurosciences of Montpellier University of Montpellier, INSERM  Montpellier France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Diab","sequence":"additional","affiliation":[{"name":"Sleep\u2010Wake Disorders Unit, Department of Neurology Gui\u2010de\u2010Chauliac Hospital, CHU Montpellier  Montpellier France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oph\u00e9lie","family":"Thobois","sequence":"additional","affiliation":[{"name":"Sleep\u2010Wake Disorders Unit, Department of Neurology Gui\u2010de\u2010Chauliac Hospital, CHU Montpellier  Montpellier France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sofiene","family":"Chenini","sequence":"additional","affiliation":[{"name":"Sleep\u2010Wake Disorders Unit, Department of Neurology Gui\u2010de\u2010Chauliac Hospital, CHU Montpellier  Montpellier France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00e9verine","family":"B\u00e9ziat","sequence":"additional","affiliation":[{"name":"Institute of Neurosciences of Montpellier University of Montpellier, INSERM  Montpellier France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Jaussent","sequence":"additional","affiliation":[{"name":"Institute of Neurosciences of Montpellier University of Montpellier, INSERM  Montpellier France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yves","family":"Dauvilliers","sequence":"additional","affiliation":[{"name":"Sleep\u2010Wake Disorders Unit, Department of Neurology Gui\u2010de\u2010Chauliac Hospital, CHU Montpellier  Montpellier France"},{"name":"National Reference Centre for Orphan Diseases, Narcolepsy, Idiopathic Hypersomnia, and Kleine\u2010Levin Syndrome  Montpellier France"},{"name":"Institute of Neurosciences of Montpellier University of Montpellier, INSERM  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