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Kleine–Levin syndrome

Long-term treatment of 15 German patients
  • G. Mayer
Schwerpunkt

Abstract

Kleine–Levin syndrome (KLS) is a rare sleep disorder. Most of the published studies have included only small numbers of patients. Therapeutic and diagnostic studies have the lowest evidence level. This study is a retrospective study including 15 KLS patients of the past 19 years. The patients had first symptoms in their early teens triggered by sleep deprivation, infections, and alcohol intake. Frequency ranged from 2–16/year, duration from 1–6 weeks. Liver enzymes, blood count, inflammatory markers, and magnetic resonance images were normal in all patients. Cerebral spinal fluid (CSF) hypocretin-1 was assessed in 6 patients. It was at the lower normal range in 2 patients and < 130 pg/ml in 2 other patients during an asymptomatic phase, while it was low in 2 patients (96–123 pg/ml) and normal in another patient during a symptomatic phase. Treatment with lithium was established in almost all patients and resulted in marked reduction or cessation of symptomatic episodes. The longest observation was 19 years with a relapse after cessation of lithium. Assessment of CSF hypocretin-1 during and outside of a symptomatic phase is recommended in the diagnostic workup of KLS; however, so far only the clinical course is helpful in establishing the diagnosis. Early treatment is recommended and has been found to be effective in the long course of the disease.

Keywords

Kleine-Levin syndrome Hypersomnia Hypocretin-1 Lithium treatment Disorders of excessive somnolence 

Kleine-Levin-Syndrom

Langzeitbehandlung 15 deutscher Patienten

Zusammenfassung

Das Kleine-Levin-Syndrom (KLS) ist eine seltene Erkrankung. Die meisten Studien haben nur kleine Fallzahlen. Therapeutische und diagnostische Studien weisen daher den niedrigsten Evidenzlevel auf. Unsere retrospektive Studie umfasst 15 KLS-Patienten der letzten 19 Jahre. Bei den Patienten traten die ersten Symptome in ihrer frühen Teenagerzeit auf, ausgelöst durch Schlafdeprivation, Infektionen und Alkoholgenuss. Die Häufigkeit symptomatischer Phasen liegt im Bereich von 2–16/Jahr, die Dauer bei 1–6 Wochen. Leberwerte, Blutbild, Entzündungsmarker und Magnetresonanztomographie waren bei allen Patienten normal. Liquor-Hypocretin-1 wurde bei 6 Patienten untersucht. In der asymptomatischen Phase war es bei 2 Patienten im unteren Normbereich und bei 2 Patienten < 130 pg/ml, bei 2 Patienten erniedrigt in der symptomatischen Phase (96–123 pg/ml) und bei einem Patienten normal. Fast alle Patienten wurden mit Lithium behandelt, das eine deutliche Reduktion oder das Sistieren der symptomatischen Phasen bewirkte. Die längste Beobachtung betrug 19 Jahre mit einem Rückfall nach dem Absetzen des Lithiums. Die Bestimmung von Liquor-Hypocretin-1 während und außerhalb der symptomatischen Phase kann als Methode bei der Diagnostik des KLS empfohlen werden, allerdings kann bisher nur der klinische Verlauf dazu beitragen, die Diagnose zu erhärten. Eine frühe Behandlung wird empfohlen. Sie scheint im Langzeitverlauf hilfreich zu sein.

Schlüsselwörter

Kleine-Levin-Syndrom Hypersomnie Hypocretin-1 im Liquor Lithiumbehandlung Störungen mit exzessiver Schläfrigkeit 

Notes

Conflict of interest

The author states that there are no conflicts of interest.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • G. Mayer

There are no affiliations available

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