Kleine-Levin syndrome: Functional imaging correlates of hypersomnia and behavioral symptoms

and APHM, CERIMED (E.G.), Aix Marseille University, Marseille, France.
Neurology (Impact Factor: 8.29). 10/2012; 79(18). DOI: 10.1212/WNL.0b013e318271f85a
Source: PubMed


Kleine-Levin syndrome (KLS) is a disorder characterized by recurrent episodes of hypersomnia associated with perception, cognitive, and behavioral disturbances, such as hyperphagia and hypersexuality.(1) Episodes are separated by intervals of normal alertness, cognition, and behavior. Primary KLS predominantly affects adolescent males, with a prevalence of 2-10 per million, although this is probably underestimated. KLS diagnosis does not rely on any reliable biological marker, while its pathophysiology remains elusive. It was suggested that KLS could result from viral or postinfectious autoimmune encephalitis(2) with a primary involvement of the hypothalamus because of the important role of this structure in regulating sleep and behavior.(1).

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    • "Hypothalamic abnormality has been suggested due to the important function of the hypothalamus in sleep regulation. Hypoperfusion or decreased metabolism have been observed in the hypothalamus in a few cases (18–21), however, hormone and hypocretin levels are usually normal. Interperiodic hypoperfusion in cerebral blood flow (CBF) demonstrated by single photon emission tomography (SPECT) is also described in frontal and temporal regions (17, 22). "
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    ABSTRACT: This work aims at reviewing the present state of the art when it comes to understanding the pathophysiology of narcolepsy and the Kleine–Levin syndrome (KLS) from a neuroimaging point of view.This work also aims at discussing future perspectives of functional neuroimag-ing in these sleep disorders. We focus on functional magnetic resonance imaging (fMRI), which is a technique for in vivo measurements of brain activation in neuronal circuitries under healthy and pathological conditions. fMRI has significantly increased the knowledge on the affected neuronal circuitries in narcolepsy and the Kleine–Levin syndrome. It has been shown that narcolepsy is accompanied with disturbances of the emotional and the closely related reward systems. In the Kleine Levin syndrome, fMRI has identified hyper-activation of the thalamus as a potential biomarker that could be used in the diagnostic procedure. The fMRI findings in both narcolepsy and the Kleine–Levin syndrome are in line with previous structural and functional imaging studies. We conclude that fMRI in combi-nation with multi-modal imaging can reveal important details about the pathophysiology in narcolepsy and the Kleine–Levin syndrome. In the future, fMRI possibly gives opportunities for diagnostic support and prediction of treatment response in individual patients.
    Frontiers in Neurology 06/2014; 5. DOI:10.3389/fneur.2014.00105
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    ABSTRACT: Objective: We report a focal morphofunctional brain impairment within the left temporopolar cortex in a patient with a posttraumatic hypersomnia. This case may contribute to better understanding the possible pathophysiological mechanism for posttraumatic hypersomnia.
    The Journal of head trauma rehabilitation 02/2013; 28(6). DOI:10.1097/HTR.0b013e3182803eda · 2.92 Impact Factor
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    ABSTRACT: The Kleine-Levin syndrome (KLS) is a rare disease which can occur one to several times per year. The KLS belongs to the group of hypersomnia of central origin occurring mainly during the second decade of life after infections, sleep deprivation, alcohol consumption or minor trauma. Early manifestation combined with hypersexuality during symptomatic phases can be a predictor for a long course of the disease, which lasts a mean of 1-27 years. Due to the lack of biological markers diagnosis at first manifestation is difficult. The classical trias of hypersomnia, hyperphagia and hypersexuality can only be found in 45 % of patients. The dominant clinical symptoms are hypersomnia with changes in perception and behavior. Subtraction of perfusion studies performed during symptomatic and asymptomatic phases showed decreased perfusion of the left hypothalamus, thalamus, basal ganglia, medial and dorsolateral frontal and temporal regions. In the few patients who had lumbar punctures in both symptomatic and asymptomatic phases hypocretin-1 was moderately to slightly lowered during symptomatic phases. Meta-analyses showed good therapeutic effects of stimulants on the symptom sleepiness. Lithium reduces the frequency and duration of symptomatic phases. Assuming that KLS is underdiagnosed it should be considered as a differential diagnosis in young patients with recurrent hypersomnia.
    Der Nervenarzt 08/2013; 84(10). DOI:10.1007/s00115-013-3858-9 · 0.79 Impact Factor
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