Central hypersomnias through the eyes of time

Keynote Speaker:
Soňa Nevšímalová, MD, DSc

Abstract Summary:
Central hypersomnias have a long tradition in our country thanks to Bedrich Roth. The first cases of idiopathic hypersomnia were described more than 60 years ago, and Prof. Roth collected in the 70s and 80s the largest clinical cohort of 1,000 patients with excessive daytime sleepiness. Later research confirmed many of his ideas. According to present views, narcolepsy type 1 is an autoimmune disease due to a focal neurodegenerative process, while narcolepsy type 2 is a less clear clinical entity. Changes in biomarkers (particularly hypocretin and histamine) together with increasing attention to children´s cases helped to improve our knowledge of narcolepsy etiology. In spite of the disappearance of secondary narcolepsy from the latest International Classification (ICSD-3), rare cases due to brain damage by metabolic disorder (particularly Niemann-Pick type C disease) and/or tumors still exist. Idiopathic hypersomnia with long nighttime sleep, disappearing from the ICDS-3 as well, seems to be another separate disease with a strong genetic predisposition worth molecular analysis. Idiopathic hypersomnia without long sleep is a little vague clinical entity reminiscent of narcolepsy type 2. Of much etiological interest is also Kleine-Levin syndrome including different phenotypes with a variety of biomarkers and therapeutic results. Hence, central hypersomnias seem to be a hot topic for a new design of ICSD-4 classification.

Professor of Neurology, Charles University, Prague, Czech Republic, Dr. Nevšímalová has devoted the greatest part of her life to sleep medicine. She has published over 200 scientific papers, 7 monographs and 32 monographic chapters. Her main interest is focused on narcolepsy and childhood sleep disorders. She received many scientific awards (including one from the American Academy of Neurology) and honorary prizes for her research. At present, she is the Vice-President of the Czech Sleep Society, and just finished a four-year period as the President of the Czech Society of Child Neurology. She has organized several International Congresses, and participated in the Scientific Committees of the ESRS and WASM. The main organizing effort is now focused on the World Sleep 2017 to be held in Prague.