Endocrine Abstracts

Introduction

Kleine-Levin syndrome, also called recurrent hypersomnia is a rare sleep disorder characterized by recurrent episodes of severe hypersomnia associated with cognitive and behavioral disturbances such as confusion, derealization, apathy, compulsive eating and hypersexuality. Menstrual-related hypersomnia is classified as a subtype of syndrome Levin-Kleine consisting of recurrent hypersomnia that is temporally linked with menses.

Case presentation

An unusual case of an 15-year-old girl with repeated episodes of hypersomnia, bradymenorrhea, behavioral disturbances similar to psychotic episodes including visual hallucinations (spiders) and anxiety. The sleep periods occurred in connection with ovulatory menstrual cycles and it was observed the absence of hallucinatory episodes during menstrual supression. Various pharmacological options have been proposed to reduce symptoms during the episodes, including anti-psychotics (haloperidol, risperidone), anti-depressants (sertraline, lorazepame), but with little amelioration on the patient’s symptoms. Her physical examinations revealed no abnormalities and on mental status examination, she appeared to be very distressed due to her symptoms. Investigation of the menstrual cycle failed to document any striking hormonal abnormality. Serum levels of follicle stimulating hormone, luteinizing hormone, estradiol and progesterone were normal. The thyroid function tests showed Hashimoto’s thyroiditis with normal thyroid hormone levels. Hormone evaluation also indicated minimal hyperprolactinemia secondary to psychiatric treatment. CSF levels of hypocretin-1, a hypothalamic peptide that has been shown to be deficient in narcolepsy was less < 30 pg/ml. A magnetic resonance imaging scan was performed and showed no cerebral abnormality. Nocturnal polysomnogram and multiple sleep latency testing were positive for narcolepsy and central sleep apnea.

Conclusion

Symptoms similarities between menstrual-related hypersomnia and other psychiatric disorders make effective diagnosis challenging. Consequently, additional clinical features and ineffective psychiatric treatment mandate further diagnoses. Sex hormones play a role in the menstrual cycle and frequently in the regulation of daily sleep-wake rhytm in women. The patient’s treatment proposed was combined oral contraceptive treatment: chlormadinone acetate and ethinylestradiol. This case underlines the importance of multidisciplinary evaluation and treatment in unusual cases.