Abstract
Introduction: Kleine–Levin syndrome (KLS) is a rare syndrome of periodic hypersomnia and behavioral and cognitive symptoms based on clinical criteria. In the setting of differential diagnosis of hypersomnia disorders, an objective diagnostic aid is desirable. A promising modality is single photon emission computed tomography (SPECT). As intraepisodal investigations are difficult to perform, an interepisodal investigation would be very helpful. Another aim of the study was to correlate SPECT findings to prognosis.Methods and Materials: Twenty-four KLS-patients were categorized as severe or non-severe based on clinical characteristics. The clinical characteristics were analyzed in relation to SPECT-examinations performed between hypersomnia periods (interepisodal) or after remission, as a clinical routine investigation.Results: Forty-eight percent of the KLS-patients have hypoperfusion in the temporal or fronto-temporal regions. In patients that have undergone remission, 56% show that pattern. There were no specific findings related to prognosis.Discussion/Conclusion: SPECT might be a diagnostic aid, in a setting of hypersomnia experience. With a sensitivity of 48%, interepisodal SPECT alone cannot be used for diagnosing KLS.
Highlights
Kleine–Levin syndrome (KLS) is a rare syndrome of periodic hypersomnia and behavioral and cognitive symptoms based on clinical criteria
The aim of this study is to elucidate whether measurements of brain perfusion is a practicable aid in diagnosing KLS between hypersomnia episodes and if specific findings are associated with severity of the disorder, i.e., we aimed to investigate if single photon emission computed tomography (SPECT) could be a prognostic aid
The present study shows that 48% of the investigated patients with KLS have abnormal perfusion between hypersomnia periods, as detected by SPECT
Summary
Kleine–Levin syndrome (KLS) is a rare syndrome of periodic hypersomnia and behavioral and cognitive symptoms based on clinical criteria. In the setting of differential diagnosis of hypersomnia disorders, an objective diagnostic aid is desirable. As intraepisodal investigations are difficult to perform, an interepisodal investigation would be very helpful Another aim of the study was to correlate SPECT findings to prognosis. The diagnosis is based on clinical criteria according to the American Academy of Sleep Medicine Classification (2005), including the cardinal symptom of periodic hypersomnia and behavioral and cognitive disturbances [1]. The rarity of the syndrome, in combination with the clinical difficulties in differentiating from other hypersomnias (such as narcolepsy, delayed sleep phase syndrome, psychiatric disorder, fatigue from infections or MS, and narcotic drug abuse) makes an objective diagnostic aid highly desirable. Another case showed normal intraepisodal orexin [5]
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