Syndrome de Kleine-Levin : complications à long terme et mécanismes des troubles cognitifs, de l'apathie et de la déréalisation

Abstract : Kleine-Levin syndrome (KLS) is a rare neuropsychiatric disease which occurs in young subjects and for wich the etiopathology is still unknown. It is characterized by recurrent episodes during several days with hypersomnia, apathy, derealization, cognitive disorders and behavioral disinhibition. Those episodes alternate with long periods of normal sleep, cognition, mood, and behavior. Are there more prolonged episodes and long-term impairment? What are the mechanisms of derealization and apathy in the KLS? To answer these questions, we used a clinical approach (interviews, questionnaires), cognitive (neuropsychological assessment) and functional brain imaging (scintigraphy) in a controlled study. Our results show that nearly one third of the patients have long episodes (over a month) and are more anxious, depressed, sleepy and tired than the others. A quarter to a third of the patients has cognitive impairment between the episodes. Cortical and subcortical hypoperfusion persists in 41 patients, especially if the duration of the episodes is long, the last episode is recent and the derealization is severe. The emergence of symptoms is associated with the hypoperfusion in the dorsal medial prefrontal cortex and in the temporoparietal junction. Those brain regions are involved in treating the information related to attention, multisensory integration, mental representations and motivation.Although the clinical expression of this disease obeys a relapsing- remitting pattern, our results suggest that those episodes could be only the tip of the "iceberg", challenging the concept of a benign disorder.
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Sophie Lavault. Syndrome de Kleine-Levin : complications à long terme et mécanismes des troubles cognitifs, de l'apathie et de la déréalisation. Neurosciences [q-bio.NC]. Université Pierre et Marie Curie - Paris VI, 2015. Français. ⟨NNT : 2015PA066592⟩. ⟨tel-01395821⟩

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