Kleine–Levin syndrome: A systematic study of 108 patients

Abstract

The similarity of the clinical and demographic features across studies strongly suggests that Kleine-Levin syndrome is a genuine disease entity. Familial clustering and increased prevalence in the Jewish population support a role for a major genetic susceptibility factor. Considering the inefficacy of available treatments, we propose that disease management should primarily be supportive and educational.

Prevalence and Mimics of Kleine-Levin Syndrome: A Survey in French-Speaking Switzerland

“…11,13,16 The significant difference regarding triggers should be viewed cautiously, as some KLS patients reported in the literature did not remember precipitant events and, given the nature of our ascertainment, a recall bias is not formally excluded. 2,13 Finally, in our center, mimic patients outnumbered KLS patients, as opposed to a recent French nationwide publication. 8 While this may reflect a referral bias or a geographical specific characteristic, it can prove relevant for practicing sleep specialists and neurologists.…”

“…[3][4][5][6][7] Several precipitating factors have been described before the occurrence of an episode, among which are infections, alcohol consumption, sleep deprivation, unusual stress, and head trauma. 2,[8][9][10][11] A study pointed to a significantly increased HLA-DQB1*0201 allele frequency in KLS patients, reinforcing the hypothesis of a possible autoimmune origin, but this has not been confirmed in a larger cohort. 2,12 To date, no placebo-controlled trials concerning treatment management are available, although lithium seems to be the most efficacious prophylactic drug for patients with frequent and disabling episodes.…”

“…2,[8][9][10][11] A study pointed to a significantly increased HLA-DQB1*0201 allele frequency in KLS patients, reinforcing the hypothesis of a possible autoimmune origin, but this has not been confirmed in a larger cohort. 2,12 To date, no placebo-controlled trials concerning treatment management are available, although lithium seems to be the most efficacious prophylactic drug for patients with frequent and disabling episodes. 2,11,13,14 The prevalence of KLS, classically estimated at 1 per million, has been recently evaluated at 1.8 cases per million in France.…”

“…2 Its pathophysiology is still unknown, although according to recent functional imaging studies, hypothalamus, thalamus, caudate nucleus, angular gyrus, anterior cingulated, dorsomedial prefrontal, orbitofrontal, and temporal cortices seem to be hypoperfused during episodes. [3][4][5][6][7] Several precipitating factors have been described before the occurrence of an episode, among which are infections, alcohol consumption, sleep deprivation, unusual stress, and head trauma.…”

“…2,12 To date, no placebo-controlled trials concerning treatment management are available, although lithium seems to be the most efficacious prophylactic drug for patients with frequent and disabling episodes. 2,11,13,14 The prevalence of KLS, classically estimated at 1 per million, has been recently evaluated at 1.8 cases per million in France. 8 At our center, we currently follow several KLS patients, which led us to suspect a higher prevalence than described in the literature.…”