Disturbed hypothalamic-pituitary axis in idiopathic recurring hypersomnia syndrome

Abstract

Disturbed function of the hypothalamic-pituitary axis at adrenal and thyroid levels was found during an episode of hypersomnia in the idiopathic recurring hypersomnia syndrome. These endocrinological abnormalities, abolished ACTH and cortisol responses to insulin-induced hypoglycemia and absent TSH response to TRH,normalised thereafter in the symptom-free interval. These data support the hypothesis that Kleine-Levin syndrome is related to an intermittent hypothalamic dysfunction.

PANDAS and Comorbid Kleine–Levin Syndrome

“…Mixed evidence suggests hypothalamic dysfunction in KLS (Koerber et al 1984;Gadoth et al 1987;Fernábdez et al 1990;Chesson et al 1991;Malhotra et al 1997;Mayer et al 1998;Kas et al 2014). Evidence for thalamic involvement also exists; Tc-99m ethyl cysteinate dimer (ECD) single-photon emission CT analysis during symptomatic periods of KLS has demonstrated thalamic hypoperfusion (Huang et al 2005;Hong et al 2006;Huang et al 2012b;Kas et al 2014).…”

First Attack of Kleine-Levin Syndrome Triggered by Influenza B Mimicking Influenza-associated Encephalopathy

“…About one-quarter of patients with KLS experience a precipitating flu-like fever before the onset and reoccurrence of the hypersomnia attack (2). A strong relationship between an upper respiratory infection and the first attack of KLS has recently been reported (3), although identification of the infectious agents has been very rare (4)(5)(6)(7)(8). The clinical profiles of 5 patients who were reported to have KLS that was triggered by identified infectious agents are summarized in Table 1.…”

“…Kleine-Levin syndrome (KLS) is a disorder that is classified as a subtype of recurrent hypersomnia and that presents with recurrent hypersomnia plus binge eating, hypersexuality, cognitive disturbances, and/or odd behavior (1,2). Various infections often precede the onset and reoccurrence of KLS (2,3), although the infectious agents have rarely been identified (4)(5)(6)(7)(8). Patients with KLS often present with cognitive disturbances, mood disorders, and irritability (1,2), and, thus, they are sometimes misdiagnosed as psychiatric disorders or encephalopathy (9).…”

Kleine-Levin-Syndrom

“…1; 14, 17). In drei Fällen mit symptomatischem KLS fanden sich strukturelle Veränderungen im Bereich des Hypothalamus (9,16).…”