title: Posterior reversible encephalopathy syndrome in a patient with mixed connective tissue disease: a case report.
abstract: BACKGROUND
  Posterior reversible encephalopathy is a syndrome highly associated with hypertension and cytotoxic therapy. The syndrome typically presents with headache, visual abnormality, seizures and characteristic vasogenic edema on magnetic resonance imaging. The entity warrants a prompt diagnosis to avoid deteriorating consequences.
  
  
  METHODS
  In this report, we describe a 15-year-old Iranian boy who was diagnosed with mixed connective tissue disease, and cyclophosphamide pulse therapy was administered. Three days after the second pulse of cyclophosphamide, when he was receiving prednisolone and hydroxycholoroquine, our patient developed generalized tonic-clonic seizures. Magnetic resonance imaging findings showed high signal intensities in the posterior areas of his brain. After 8 days, the brain magnetic resonance imaging abnormalities were resolved following the control of his blood pressure and antiepileptic treatment. These observations have been indicative of posterior reversible encephalopathy syndrome. Nevertheless, our patient developed uncontrollable respiratory distress and eventually died.
  
  
  CONCLUSIONS
  To the best of our knowledge, this case is the first report of posterior reversible encephalopathy syndrome in a patient with mixed connective tissue disease. As the patient developed posterior reversible encephalopathy syndrome 3 days after cyclophosphamide pulse therapy to reduce the disease activity, it is hard to accurately determine whether posterior reversible encephalopathy syndrome in this case is a complication of cyclophosphamide or a condition that resulted from the mixed connective tissue disease flare-up.
authors.affiliation: Division of Neuroscience, Cellular and Molecular Research Center, Iran University of Medical Sciences, Tehran, Iran.
authors.forename: Reza
authors.initials: R
authors.lastname: Rahmanzadeh
authors.affiliation: Division of Neuroscience, Neurology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: Ramin
authors.initials: R
authors.lastname: Rahmanzade
authors.affiliation: Bone and Joint Reconstruction Research Center, Shafa Orthopedic Hospital, Iran University of Medical Sciences, Tehran, Iran. Mozhdehzabihi@gmail.com.
authors.forename: Mozhdeh
authors.initials: M
authors.lastname: Zabihiyeganeh
mesh_terms: D000293:Adolescent; D001921:Brain / Q000000981:diagnostic imaging; D003520:Cyclophosphamide / Q000009:adverse effects*; D017809:Fatal Outcome; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects*; D008279:Magnetic Resonance Imaging; D008297:Male; D008947:Mixed Connective Tissue Disease / Q000188:drug therapy*; D054038:Posterior Leukoencephalopathy Syndrome / Q000139:chemically induced* / Q000000981:diagnostic imaging; D012640:Seizures / Q000139:chemically induced
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide
keywords: Cyclophosphamide; Mixed connective tissue disease; Posterior reversible encephalopathy syndrome