Kleine-Levin syndrome (KLS) is a rare sleep disorder characterized by periodic hypersomnia and various degrees of cognitive and behavioral disturbance, hyperphagia, and hypersexuality. Effective treatment is challenging. Stimulants marginally address sleepiness, but may increase irritability and do not improve cognitive and behavioral disturbances. Modafi nil may shorten the symptomatic period but not the recurrence rate. Lithium and carbamazepine are benefi cial in some cases, possibly related to similarities between KLS and affective disorders. Currently, no single medication is consistently successful in treating the syndrome. Here we report the shortterm effect of clarithromycin in a patient with KLS.
C A S E R E P O R T SK leine-Levin Syndrome (KLS) is a rare sleep disorder of unknown cause characterized by repetitive, intermittent cycles of extreme sleepiness, and cognitive/behavioral disturbances including confusion, feelings of unreality, aggressiveness, and hypersexuality. Gamma-aminobutyric acid (GABA) is the predominant inhibitory neurotransmitter in the brain with well-known effects on sleep/wake cycle regulation. The GABA A receptor is the site where sleep-inducing drugs such as diazepam have their effects, and recent research suggests GABA A receptor antagonists may be useful in the treatment of hypersomnia.1 We sought to learn if clarithromycin, an antibiotic with GABA A receptor antagonistic properties, would be benefi cial in the treatment of KLS.
REPORT OF CASEWe report a 23-year-old female with KLS beginning at 10 years of age. Her diagnosis was consistent with ICSD-2 criteria and based on recurrent symptoms of hypersomnia, hypersexuality, irritability, and child-like behavior with a dreamlike state.2 KLS episode triggers included alcohol, upper respiratory infections, and general anesthesia. She slept 14 to 18 h/night during hypersomnia episodes, which could last from weeks to months. She typically had headache, nausea, and vomiting at episode onset. MRI perfusion showed episode related reductions in thalamic perfusion.3 Episode length and duration were variable, but over time they had decreased in severity allowing performance of some of her daily life functions. Between episodes her sleep duration was approximately 9 h/night with normal mood and activity levels. She had no comorbid illness and took no regular medications. She had tried a number of medications for KLS, including prazosin, acetazolamide, methylphenidate, and liothyronine, all of which were discontinued due to either side effects or ineffectiveness. In the midst of a recent episode she had been started on clarithromycin 500 mg every 12 hours. She was on no other medications at that time. Within 24 h, her sleepiness