title: Sporadic Creutzfeldt-Jakob disease with unusual initial presentation as posterior reversible encephalopathy syndrome: a case report.
abstract: BACKGROUND
  Creutzfeldt - Jakob disease (CJD) is a rapidly progressive and fatal neurodegenerative prion disease. MRI findings are included in diagnostic criteria for probable CJD, giving a sensitivity and specificity more than 90%, but the atypical radiological presentations in the early stage of the disease could cause the diagnostic difficulties. CJD can be definitively diagnosed by histopathological confirmation, brain biopsy or at autopsy.
  
  
  METHODS
  We present a case of 53-year-old woman with a history of a rapidly progressive dementia with symptoms of visual impairment, increased extrapyramidal type muscle tonus, stereotypical movements and ataxic gait resulting in the patient's death after13 months. The clinical symptoms deteriorated progressively to myoclonus and akinetic mutism already on the 14th week. The series of diagnostic examinations were done to exclude the possible causes of dementia. Initial MRI evaluation as posterior reversible encephalopathy syndrome (PRES) on the 9th week after the onset of symptoms created us a diagnostic conundrum. Subsequent MRI findings of symmetrical lesions in the basal ganglia (nucleus caudatus, putamen) on the 13th week and EEG with periodic sharp wave complexes (PSWC) in frontal regions on the 18th week allowed us to diagnose the probable sCJD. The histopathological findings after brain biopsy on the 14th week demonstrated the presence of the abnormal prion protein deposits in the grey matter by immunohistochemistry with ICSM35, KG9 and 12 F10 antibodies and confirmed the diagnosis of sCJD.
  
  
  CONCLUSIONS
  In this article we focus our attention on a rare association between radiological PRES syndrome and early clinical stage of sCJD. Although concurrent manifestation of these conditions can be accidental, but the immunogenic or neuropeptide mechanisms could explain such radiological MRI findings. A thorough knowledge of differential diagnostic of PRES may be especially useful in earlier diagnosis of sCJD.
authors.affiliation: Department of Psychiatry, Lithuanian University of Health Sciences, Mickeviciaus str. 9, Kaunas, LT-44307, Lithuania. edgarasdirzius@gmail.com.
authors.forename: Edgaras
authors.initials: E
authors.lastname: Dirzius
authors.affiliation: Department of Neurology Lithuanian University of Health Sciences, Mickeviciaus str. 9, Kaunas, LT-44307, Lithuania.
authors.forename: Renata
authors.initials: R
authors.lastname: Balnyte
authors.affiliation: Department of Psychiatry, Lithuanian University of Health Sciences, Mickeviciaus str. 9, Kaunas, LT-44307, Lithuania.
authors.forename: Vesta
authors.initials: V
authors.lastname: Steibliene
authors.affiliation: Department of Radiology Lithuanian University of Health Sciences, Mickeviciaus str. 9, Kaunas, LT-44307, Lithuania.
authors.forename: Rymante
authors.initials: R
authors.lastname: Gleizniene
authors.affiliation: Department of Pathology Lithuanian University of Health Sciences, Mickeviciaus str. 9, Kaunas, LT-44307, Lithuania.
authors.forename: Inga
authors.initials: I
authors.lastname: Gudinaviciene
authors.affiliation: Department of Neurosurgery Lithuanian University of Health Sciences, Mickeviciaus str. 9, Kaunas, LT-44307, Lithuania.
authors.forename: Andrius
authors.initials: A
authors.lastname: Radziunas
authors.affiliation: Department of Neurology Lithuanian University of Health Sciences, Mickeviciaus str. 9, Kaunas, LT-44307, Lithuania.
authors.forename: Kestutis
authors.initials: K
authors.lastname: Petrikonis
mesh_terms: D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications / Q000000981:diagnostic imaging*; D003704:Dementia / Q000209:etiology*; D003951:Diagnostic Errors; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008875:Middle Aged; D054038:Posterior Leukoencephalopathy Syndrome / Q000175:diagnosis*
keywords: Biopsy; Case report; Magnetic resonance imaging; Posterior reversible encephalopathy syndrome; Sporadic Creutzfeldt-Jakob disease