Abstract

Abstract Introduction Kleine-Levin Syndrome (KLS) is a rare disorder of hypersomnolence with cognitive and behavioral disturbances. Currently, there are no definitive treatment recommendations although stimulants, mood stabilizers, and even electroconvulsive therapy have been utilized with inconsistent results. Report of Cases: A 23-year-old female with a history of depression, anxiety, obsessive-compulsive disorder, Hashimoto's thyroiditis, lupus, and morbid obesity presented to our facility for management of debilitating episodes of sleepiness due to KLS, diagnosed at the age of 22. She reported good health until a series of viral illnesses at the age of 20. She then began experiencing sleep episodes requiring 20-22 hours of sleep per day, only awakening to eat and use the bathroom. These episodes typically lasted for two weeks and were preceded by periods of hyperphagia and hypersexuality. When she presented to our facility, she was using lithium and modafinil daily along with amantadine and clarithromycin twice daily as needed for episodes of sleepiness. At her initial visit, she was prescribed transdermal flumazenil, a GABA-A receptor/benzodiazepine antagonist, to be used as needed to supplement amantadine and clarithromycin. She was initially requiring flumazenil every other month. After seven months of flumazenil use, the frequency of episodes decreased to every 3-4 months. She would administer two clicks of flumazenil cream on each forearm with the prodrome symptom of fogginess, which would abort the episode. Rarely, she required a second dose 12 hours later. With flumazenil and avoidance of triggers, such as alcohol and sleep deprivation, she reported completing her master’s degree and finally maintaining a job. Ultimately, she discontinued lithium, modafinil, amantadine, and clarithromycin use without reoccurrence of her symptoms. Conclusion This case demonstrates successful treatment of KLS with the use of a GABA-A receptor/benzodiazepine antagonist, flumazenil. Reduction in episodes of sleepiness with flumazenil ultimately led to discontinuing stimulant, mood stabilizer, and clarithromycin use. Early trials of transdermal flumazenil and continued research regarding the use of this medication for patients with KLS may prove to be a promising intervention. Support (If Any) None.

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