Greater reduction of striatal dopamine transporter availability during the symptomatic than asymptomatic phase of Kleine-Levin syndrome.

摘要

Kleine-Levin syndrome (KLS) is a rare disorder characterized by recurrent periods of hypersomnia, compulsive hyperphagia, hyper-sexuality and other behavioural/cognitive disturbances [1]. Although its exact physiopathology remains elusive, several lines of evidence point to an involvement of the dopaminergic system [2-4]. We previously demonstrated reduced Dopamine Transporter (DAT) availability in the asymptomatic phase of KLS as compared to controls [4]. Herein, after approval by the Ethics Committee at the Universidade Federal de Sao Paulo, we performed brain Single Photon Computed Tomography (SPECT) imaging with [Tc-99m]-TRODAT-l to assess in vivo DAT availability between the symptomatic phase of KLS vs. the asymptomatic phase in the same patient and in control subjects (n = 7) matched for gender (male) and age (mean age: 20.8 years; SD: +-2.8). Our analyses were based on an automatic 3D volumetric striatal region of interest quantification reported elsewhere [5]. Bilateral striatum DAT binding potential (BP) was reduced in both symptomatic (left striatum DAT-BP: 1.18; right striatum DAT-BP: 1.07) and asymptomatic (left striatum DAT-BP: 1.22; right striatum DAT-BP: 1.22) KLS compared to controls (mean left striatum DAT-BP: 1.28; SD: +-0.10; mean right striatum DAT-BP: 1.30; SD: +-0.11).