A strange New Year's Eve: triggers in Kleine-Levin syndrome

Abstract

Kleine-Levin syndrome is a rare neurological disease of unknown cause beginning typically during adolescence, characterized by remittent-relapsing episodes of severe hypersomnia associated with cognitive and behavioral disturbances. Triggering factors at Kleine-Levin syndrome onset include infection, sleep deprivation, as well as alcohol, drug, and substance intake. A young woman had 6 episodes over 2 years, including hypersomnia, confusion, derealization, cognitive impairment, anxiety, feeling of being scrutinized, anorexia (and sweet craving once) but no hypersexuality. The first episode started after a party where she experienced a complete, 4-hour-long blackout despite moderate alcohol intake. The patient suspected having been poisoned. Twenty-five months after the party, when Kleine-Levin syndrome was eventually diagnosed, her long hair was analyzed and exogenous γ-hydroxybutyrate was found in the tips (corresponding to the party time). This case illustrates the interest of looking for γ-hydroxybutyrate in the hair when Kleine-Levin syndrome starts after a party.

Keywords: Kleine-Levin syndrome; gamma-hydroxybutyrate (GHB); hypersomnia; poisoning.

Figure 1. Brain imaging.

Functional brain imaging (using 18-fluorodeoxyglucose positron emission tomography) during an asymptomatic period in the patient with KLS. (A) Axial slice showing mild bilateral hypometabolism in the posterior associative temporo-occipital cortex. (B) Frontal slice showing bilateral hippocampal hypometabolism. KLS = Kleine-Levin syndrome.

Figure 2. GHB concentrations in hair segments.

GHB concentration (y axis) according to function of the hair segment closer to the root (point A on the x axis) to tips (point M on x axis). New Year’s Eve should correspond to several segments between I and M on the x axis. The single poisoning is observed in several segments. Indeed, after a single poisoning, a single segment is usually raised when the hair is removed 1 month after administration. When sampling hair 2 years later, even after a single poisoning, concentrations in some segments surrounding the date of poisoning are high due to the different phases of hair growth (anagen, catagen, and telogen). GHB = γ-hydroxybutyrate.