An unusual occurrence of Kleine-Levin syndrome in a man with refractory immune thrombocytopenic purpura: a case report

Abstract

IntroductionKleine-Levin syndrome is an extremely rare neurological entity characterized by recurrent episodes of hypersomnia which are sometimes associated with compulsive hyperphagia and behavioral changes. Autoimmunity has recently been proposed as a factor contributing to its pathogenesis. Immune thrombocytopenic purpura is a relatively common autoimmune disease showing a lot of complexity and uncertainty regarding its treatment regimens and its refractory nature in some cases.Case presentationA 32-year-old Persian White man visited his private hematologist complaining of recent episodes of epistaxis and appearance of petechial lesions 24 hours after receiving a meningococcal vaccine. He had a history of immune thrombocytopenic purpura 13 years before his presentation. Based on his history and laboratory findings, his condition was diagnosed as a relapse of immune thrombocytopenic purpura and was managed accordingly. He did not respond to first-line corticosteroid regimens and later developed neurological symptoms as recurrent episodes of hypersomnia and hyperphagia. After a complete clinical and paraclinical evaluation and ruling out other possible conditions, he was given a diagnosis of Kleine-Levin syndrome. He was followed up for his immune thrombocytopenic purpura and received different treatment regimens none of which were adequately successful except intravenous immunoglobulin that was only temporarily effective. He has had 4 documented self-limited episodes of Kleine-Levin syndrome since his initial presentation.ConclusionsImmune thrombocytopenic purpura may be associated with meningococcal vaccination in adulthood. Responses to treatment in immune thrombocytopenic purpura vary among patients. Our patient only had a transient acceptable response to intravenous immunoglobulin while all other options failed to improve his platelet count. Concurrence of immune thrombocytopenic purpura and Kleine-Levin syndrome supports the role of autoimmunity as the proposed pathophysiological mechanism of Kleine-Levin syndrome.

Kleine-Levin Syndrome in a Young Woman Triggered in Travel: A Case Report From Iran in COVID-19 Arena

“…Given the role of autoimmunity in the pathology of KLS, 12 it is possible that rheumatoid arthritis in this case has exacerbated the symptoms of this syndrome. However, more studies are required to diagnose and treat this syndrome.…”

Síndrome de Kleine-Levin: diagnóstico diferencial en los síndromes encefalíticos recurrentes del adolescente

“…En la cohorte de Huang et al, hasta un 96,6% se produce tras infecciones respiratorias [7], pero también se ha descrito tras la ingesta de alcohol, la privación de sueño, traumatismos y anestesia [8,9]. No hemos encontrado ningún otro caso tras la vacuna DT, como en el caso 3, pero hay descrito uno tras la vacuna del meningococo, que desencadena a su vez una púrpura trombocitopénica, un síndrome de Kleine-Levin y otros tres casos sin especificar [10,11].…”