title: An unusual occurrence of Kleine-Levin syndrome in a man with refractory immune thrombocytopenic purpura: a case report.
abstract: BACKGROUND
  Kleine-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.
  
  
  METHODS
  A 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.
  
  
  CONCLUSIONS
  Immune 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.
authors.affiliation: Department of Neurology, Zahedan University of Medical Sciences, Zahedan, Iran. dr.amirifard@gmail.com.
authors.forename: Hamed
authors.initials: H
authors.lastname: Amirifard
authors.affiliation: Students' Scientific Research Center, Zahedan University of Medical Sciences, Zahedan, Iran. barzkarfarzane@gmail.com.
authors.forename: Farzaneh
authors.initials: F
authors.lastname: Barzkar
authors.affiliation: Department of Internal Medicine, School of Medicine, Zahedan University of Medical Sciences, Zahedan, Iran. sa.fazeli@gmail.com.
authors.forename: Seyed Amirhossein
authors.initials: SA
authors.lastname: Fazeli
authors.affiliation: Department of Hematology, Zahedan University of Medical Sciences, Zahedan, Iran. hashemimahdi@zaums.ac.ir.|Ali-Ebne-Abitaleb hospital, Zahedan, Iran. hashemimahdi@zaums.ac.ir.
authors.forename: Seyed Mehdi
authors.initials: SM
authors.lastname: Hashemi
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D006970:Disorders of Excessive Somnolence / Q000209:etiology; D006801:Humans; D006963:Hyperphagia / Q000209:etiology; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D017593:Kleine-Levin Syndrome / Q000175:diagnosis / Q000209:etiology*; D008297:Male; D022401:Meningococcal Vaccines / Q000009:adverse effects*; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000150:complications* / Q000188:drug therapy
chemical_list: D016756:Immunoglobulins, Intravenous; D022401:Meningococcal Vaccines