1152 A Journey Through Kleine-Levin Syndrome: A Longitudinal Case Report

Introduction Kleine-Levin Syndrome (KLS) is a rare disease with intermittent episodes of hypersomnia with behavioral abnormalities. We present a case of KLS with successful symptomatic treatment with modafinil and eventual remission. Report of case(s) A 19-year-old healthy male with no prior sleep disorders was referred for evaluation of hypersomnia. He developed leg cellulitis requiring antibiotic therapy. Shortly afterwards, he developed abnormal behaviors --quitting school, estrangement from friends, sleeping 20 hours a day, irritability, and suppressed appetite. He also demonstrated hypersexuality and disinhibited behaviors. After 10 days, he abruptly returned to baseline and had limited recall of the prior events. Three months later, he had a second episode with similar behavioral and sleep changes, again followed by spontaneous return to baseline after 10 days. Two months later, a similar spell occurred, prompting arrangement for psychiatric admission and initiation of quetiapine for a proposed diagnosis of rapid-cycling bipolar disorder. His symptoms again resolved spontaneously. Diagnostic tests including liver function tests, thyroid function tests, ammonia level, urine drug screen, Lyme disease, MRI brain, EEG, and CSF analysis were unremarkable. He was eventually referred to the Sleep Medicine clinic for evaluation. There were no symptoms suggesting other comorbid sleep disorders, and he had a normal neurologic and physical exam at time of presentation. A clinical diagnosis of KLS was made. The patient and family deferred prophylactic or abortive treatment (such as lithium, carbamazepine, or steroids) due to potential for side effects. Modafinil provided symptomatic benefits during the three milder episodes he had over the next few years. Episodes subsequently resolved and the patient is currently in remission (over 5 years) without sequalae. Conclusion KLS is a rare disease with typical onset during young adulthood, manifesting with abrupt onset of recurrent episodes of hypersomnia and prolonged sleep duration, lasting from days to several weeks. The diagnosis is clinical and requires at least one additional feature including: cognitive dysfunction, derealization, major apathy, or disinhibited behaviors. Patients revert to baseline between the episodes. Stimulants such as modafinil can promote wakefulness during the episodes. Support (if any) N/A