{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,10]],"date-time":"2026-11-10T15:24:34Z","timestamp":1794324274581,"version":"4.9.0"},"reference-count":116,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["FOC"],"published-print":{"date-parts":[[2015,11]]},"abstract":"<jats:p>Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative condition with a rapid disease course and a mortality rate of 100%. Several forms of the disease have been described, and the most common is the sporadic type. The most challenging aspect of this disease is its diagnosis\u2014the gold standard for definitive diagnosis is considered to be histopatho-logical confirmation\u2014but newer tests are providing means for an antemortem diagnosis in ways less invasive than brain biopsy. Imaging studies, electroencephalography, and biomarkers are used in conjunction with the clinical picture to try to make the diagnosis of CJD without brain tissue samples, and all of these are reviewed in this article. The current diagnostic criteria are limited; test sensitivity and specificity varies with the genetics of the disease as well as the clinical stage. Physicians may be unsure of all diagnostic testing available, and may order outdated tests or prematurely request a brain biopsy when the diagnostic workup is incomplete. The authors review CJD, discuss the role of brain biopsy in this patient population, provide a diagnostic pathway for the patient presenting with rapidly progressive dementia, and propose newer diagnostic criteria.<\/jats:p>","DOI":"10.3171\/2015.8.focus15328","type":"journal-article","created":{"date-parts":[[2015,11,1]],"date-time":"2015-11-01T02:36:31Z","timestamp":1446345391000},"page":"E2","source":"Crossref","is-referenced-by-count":142,"title":["Creutzfeldt-Jakob disease: updated diagnostic criteria, treatment algorithm, and the utility of brain biopsy"],"prefix":"10.3171","volume":"39","author":[{"given":"Marc","family":"Manix","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Louisiana State University Health Sciences Center, Shreveport, Louisiana"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piyush","family":"Kalakoti","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Louisiana State University Health Sciences Center, Shreveport, Louisiana"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miriam","family":"Henry","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Louisiana State University Health Sciences Center, Shreveport, Louisiana"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jai","family":"Thakur","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Louisiana State University Health Sciences Center, Shreveport, Louisiana"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Menger","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Louisiana State University Health Sciences Center, Shreveport, Louisiana"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bharat","family":"Guthikonda","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Louisiana State University Health Sciences Center, Shreveport, Louisiana"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anil","family":"Nanda","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Louisiana State University Health Sciences Center, Shreveport, Louisiana"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1-focus15328","series-title":"Nat Med","first-page":"175","article-title":"Ultrasensitive human prion detection in cerebrospinal fluid by real-time quaking-induced conversion","volume":"17","author":"Atarashi","year":"2011"},{"key":"b2-focus15328","series-title":"Neurosurgery","first-page":"283","article-title":"Diagnostic value and safety of brain biopsy in patients with cryptogenic neurological disease: a systematic review and meta-analysis of 831 cases","volume":"77","author":"Bai","year":"2015"},{"key":"b3-focus15328","series-title":"AJR Am J Roentgenol","first-page":"137","article-title":"MR diagnosis of Creutzfeldt-Jakob disease: significance of high signal intensity of the basal ganglia","volume":"162","author":"Barboriak","year":"1994"},{"key":"b4-focus15328","series-title":"BMC Infect Dis","first-page":"26","article-title":"Epidemiological evidence of higher susceptibility to vCJD in the young","volume":"4","author":"Bo\u00eblle","year":"2004"},{"key":"b5-focus15328","series-title":"WHO Manual for Surveillance of Human Transmissible Spongiform Encephalopathies, including Variant Creutzfeldt-Jakob Disease","author":"Brown","year":"2003"},{"key":"b6-focus15328","series-title":"Neurology","first-page":"1075","article-title":"Iatrogenic Creutzfeldt-Jakob disease at the millennium","volume":"55","author":"Brown","year":"2000"},{"key":"b7-focus15328","series-title":"Nature","first-page":"577","article-title":"Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein","volume":"356","author":"B\u00fceler","year":"1992"},{"key":"b8-focus15328","series-title":"J Neuroimaging","first-page":"2","article-title":"The role of neuroimaging in evaluating patients affected by Creutzfeldt-Jakob disease: a systematic review of the literature","volume":"25","author":"Caobelli","year":"2015"},{"key":"b9-focus15328","series-title":"Neurology","first-page":"436","article-title":"Sensitivity of 14\u20133-3 protein test varies in subtypes of sporadic Creutzfeldt-Jakob disease","volume":"63","author":"Castellani","year":"2004"},{"key":"b10-focus15328","series-title":"MMWR Morb Mortal Wkly Rep","first-page":"1066","article-title":"Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts -- Japan, January 1979-May 1996","volume":"46","author":"Centers for Disease Control and Prevention","year":"1997"},{"key":"b11-focus15328","series-title":"Ann Neurol","first-page":"437","article-title":"Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease","volume":"70","author":"Chitravas","year":"2011"},{"key":"b12-focus15328","series-title":"J Neurol Neurosurg Psychiatry","first-page":"1243","article-title":"The role of cerebrospinal fluid 14\u20133-3 and other proteins in the diagnosis of sporadic Creutzfeldt-Jakob disease in the UK: a 10-year review","volume":"81","author":"Chohan","year":"2010"},{"key":"b13-focus15328","series-title":"AJNR Am J Neuroradiol","first-page":"1560","article-title":"Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases","volume":"24","author":"Collie","year":"2003"},{"key":"b14-focus15328","series-title":"Z Gesamte Neurol Psychiatr","first-page":"1","article-title":"Uber eine eigenartige herdformige Erkrankung des Zentralnervensystems","volume":"57","author":"Creutzfeldt","year":"1920"},{"key":"b15-focus15328","series-title":"N Engl J Med","first-page":"692","article-title":"Possible person-to-person transmission of 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Neurosurg","first-page":"735","article-title":"Brain biopsy in patients with suspected Creutzfeldt-Jakob disease","volume":"109","author":"Heinemann","year":"2008"},{"key":"b21-focus15328","series-title":"J Pathol","first-page":"134","article-title":"Pathogenesis and prevalence of variant Creutzfeldt-Jakob disease","volume":"208","author":"Hilton","year":"2006"},{"key":"b22-focus15328","series-title":"Z Ges Neurol Psychiatry","first-page":"147","article-title":"Uber eigenartige Erkrankungen des Zentralnervensystems mit bemerkenswerten anatomischen Befunde (spastische Pseudosklerose-Encephalomyelopathie mit dissminierten Degeneratiosherden)","volume":"64","author":"Jakob","year":"1921"},{"key":"b23-focus15328","series-title":"N Engl J Med","first-page":"1994","article-title":"Creutzfeldt-Jakob disease and related transmissible spongiform encephalopathies","volume":"339","author":"Johnson","year":"1998"},{"key":"b24-focus15328","series-title":"AJNR Am J 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Neurol","first-page":"951","article-title":"Diffusion-weighted magnetic resonance imaging in probable Creutzfeldt-Jakob disease: a clinical-anatomic correlation","volume":"56","author":"Na","year":"1999"},{"key":"b38-focus15328","series-title":"Brain","first-page":"519","article-title":"Subacute spongiform encephalopathy\u2014a subacute form of encephalopathy attributable to vascular dysfunction (spongiform cerebral atrophy)","volume":"83","author":"Nevin","year":"1960"},{"key":"b39-focus15328","series-title":"N Engl J Med","first-page":"519","article-title":"A test for Creutzfeldt-Jakob disease using nasal brushings","volume":"371","author":"Orr\u00fa","year":"2014"},{"key":"b40-focus15328","series-title":"Neurology","first-page":"714","article-title":"Efficacy of flupirtine on cognitive function in patients with CJD: A double-blind study","volume":"62","author":"Otto","year":"2004"},{"key":"b41-focus15328","series-title":"Ann Neurol","first-page":"224","article-title":"Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects","volume":"46","author":"Parchi","year":"1999"},{"key":"b42-focus15328","series-title":"Folia Neuropathol","first-page":"20","article-title":"Molecular pathology, classification, and diagnosis of sporadic human prion disease variants","volume":"50","author":"Parchi","year":"2012"},{"key":"b43-focus15328","series-title":"Science","first-page":"136","article-title":"Novel proteinaceous infectious particles cause scrapie","volume":"216","author":"Prusiner","year":"1982"},{"key":"b44-focus15328","series-title":"Proc Natl Acad Sci U S A","first-page":"13363","article-title":"Prions","volume":"95","author":"Prusiner","year":"1998"},{"key":"b45-focus15328","series-title":"Proc Natl Acad Sci U S A","first-page":"3501","article-title":"Diagnosis of human prion disease","volume":"102","author":"Safar","year":"2005"},{"key":"b46-focus15328","series-title":"Acta Neuropathol","first-page":"327","article-title":"Brain biopsy in dementia: clinical indications and diagnostic approach","volume":"120","author":"Schott","year":"2010"},{"key":"b47-focus15328","series-title":"Neurology","first-page":"419","article-title":"Open biopsy in patients with acute progressive neurologic decline and absence of mass lesion","volume":"75","author":"Schuette","year":"2010"},{"key":"b48-focus15328","series-title":"Klin Wochenschr","first-page":"1817","article-title":"Die histopathologische Forschung in der Psychiatrie","volume":"1","author":"Spielmeyer","year":"1922"},{"key":"b49-focus15328","series-title":"Eff Clin Pract","first-page":"143","article-title":"The healthcare cost and utilization project: an overview","volume":"5","author":"Steiner","year":"2002"},{"key":"b50-focus15328","series-title":"Brain","first-page":"3051","article-title":"Cerebrospinal fluid biomarker supported diagnosis of Creutzfeldt-Jakob disease and rapid dementias: a longitudinal multicentre study over 10 years","volume":"135","author":"Stoeck","year":"2012"},{"key":"b51-focus15328","series-title":"Ann Neurol","first-page":"405","article-title":"Virus-induced electrotonic coupling: hypothesis on the mechanism of periodic EEG discharges in Creutzfeldt-Jakob disease","volume":"10","author":"Traub","year":"1981"},{"key":"b52-focus15328","series-title":"Public Health Service Interagency Coordinating Committee Report on Human Growth Hormone and CJD","first-page":"1","volume":"14","author":"United States Public Health Service","year":"1997"},{"key":"b53-focus15328","series-title":"Neurology","first-page":"1711","article-title":"Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias","volume":"76","author":"Vitali","year":"2011"},{"key":"b54-focus15328","series-title":"Clin Neurophysiol","first-page":"935","article-title":"EEG in Creutzfeldt-Jakob disease","volume":"117","author":"Wieser","year":"2006"},{"key":"b55-focus15328","series-title":"Clin Neurophysiol","first-page":"2467","article-title":"Serial EEG findings in sporadic and iatrogenic Creutzfeldt-Jakob disease","volume":"115","author":"Wieser","year":"2004"},{"key":"b56-focus15328","series-title":"Lancet","first-page":"921","article-title":"A new variant of Creutzfeldt-Jakob disease in the UK","volume":"347","author":"Will","year":"1996"},{"key":"b57-focus15328","series-title":"global Surveillance, Diagnosis, and Therapy of Human Transmissible Spongiform Encephalopathies: Report of a WHO Consultation","author":"World Health Organization","year":"1998"},{"key":"b58-focus15328","series-title":"Ann Neurol","first-page":"323","article-title":"Current clinical diagnosis in Creutzfeldt-Jakob disease: identification of uncommon variants","volume":"48","author":"Zerr","year":"2000"},{"key":"b1-focus15328","series-title":"Nat Med","first-page":"175","article-title":"Ultrasensitive human prion detection in cerebrospinal fluid by real-time quaking-induced conversion","volume":"17","author":"Atarashi","year":"2011"},{"key":"b2-focus15328","series-title":"Neurosurgery","first-page":"283","article-title":"Diagnostic value and safety of brain biopsy in patients with cryptogenic neurological disease: a systematic review and meta-analysis of 831 cases","volume":"77","author":"Bai","year":"2015"},{"key":"b3-focus15328","series-title":"AJR Am J Roentgenol","first-page":"137","article-title":"MR diagnosis of Creutzfeldt-Jakob disease: significance of high signal intensity of the basal ganglia","volume":"162","author":"Barboriak","year":"1994"},{"key":"b4-focus15328","series-title":"BMC Infect Dis","first-page":"26","article-title":"Epidemiological evidence of higher susceptibility to vCJD in the young","volume":"4","author":"Bo\u00eblle","year":"2004"},{"key":"b5-focus15328","series-title":"WHO Manual for Surveillance of Human Transmissible Spongiform Encephalopathies, including Variant Creutzfeldt-Jakob Disease","author":"Brown","year":"2003"},{"key":"b6-focus15328","series-title":"Neurology","first-page":"1075","article-title":"Iatrogenic Creutzfeldt-Jakob disease at the millennium","volume":"55","author":"Brown","year":"2000"},{"key":"b7-focus15328","series-title":"Nature","first-page":"577","article-title":"Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein","volume":"356","author":"B\u00fceler","year":"1992"},{"key":"b8-focus15328","series-title":"J Neuroimaging","first-page":"2","article-title":"The role of neuroimaging in evaluating patients affected by Creutzfeldt-Jakob disease: a systematic review of the literature","volume":"25","author":"Caobelli","year":"2015"},{"key":"b9-focus15328","series-title":"Neurology","first-page":"436","article-title":"Sensitivity of 14\u20133-3 protein test varies in subtypes of sporadic Creutzfeldt-Jakob 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Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases","volume":"24","author":"Collie","year":"2003"},{"key":"b14-focus15328","series-title":"Z Gesamte Neurol Psychiatr","first-page":"1","article-title":"Uber eine eigenartige herdformige Erkrankung des Zentralnervensystems","volume":"57","author":"Creutzfeldt","year":"1920"},{"key":"b15-focus15328","series-title":"N Engl J Med","first-page":"692","article-title":"Possible person-to-person transmission of Creutzfeldt-Jakob disease","volume":"290","author":"Duffy","year":"1974"},{"key":"b16-focus15328","series-title":"Br Med Bull","first-page":"213","article-title":"Sporadic and familial CJD: classification and characterisation","volume":"66","author":"Gambetti","year":"2003"},{"key":"b17-focus15328","series-title":"J Neurol","first-page":"1440","article-title":"Diffusion-weighted MRI findings and clinical correlations in sporadic Creutzfeldt-Jakob disease","volume":"262","author":"Gao","year":"2015"},{"key":"b18-focus15328","series-title":"Eur J Neurol","first-page":"121","article-title":"CSF analysis in patients with sporadic CJD and other transmissible spongiform encephalopathies","volume":"14","author":"Green","year":"2007"},{"key":"b19-focus15328","series-title":"Neuropathology","first-page":"221","article-title":"Human prion diseases: molecular, cellular and population biology","volume":"33","author":"Head","year":"2013"},{"key":"b20-focus15328","series-title":"J Neurosurg","first-page":"735","article-title":"Brain biopsy in patients with suspected Creutzfeldt-Jakob disease","volume":"109","author":"Heinemann","year":"2008"},{"key":"b21-focus15328","series-title":"J Pathol","first-page":"134","article-title":"Pathogenesis and prevalence of variant Creutzfeldt-Jakob disease","volume":"208","author":"Hilton","year":"2006"},{"key":"b22-focus15328","series-title":"Z Ges Neurol Psychiatry","first-page":"147","article-title":"Uber eigenartige Erkrankungen des Zentralnervensystems mit bemerkenswerten anatomischen Befunde (spastische Pseudosklerose-Encephalomyelopathie mit dissminierten Degeneratiosherden)","volume":"64","author":"Jakob","year":"1921"},{"key":"b23-focus15328","series-title":"N Engl J Med","first-page":"1994","article-title":"Creutzfeldt-Jakob disease and related transmissible spongiform encephalopathies","volume":"339","author":"Johnson","year":"1998"},{"key":"b24-focus15328","series-title":"AJNR Am J Neuroradiol","first-page":"1459","article-title":"Creutzfeldt-Jakob disease: comparative analysis of MR imaging sequences","volume":"27","author":"Kallenberg","year":"2006"},{"issue":"Suppl 1","key":"b25-focus15328","series-title":"J Neurol Neurosurg Psychiatry","first-page":"i36","article-title":"Prion diseases","volume":"75","author":"Knight","year":"2004"},{"key":"b26-focus15328","series-title":"Arch Neurol","first-page":"876","article-title":"Clinical features and diagnosis of the MM2 cortical subtype of sporadic Creutzfeldt-Jakob disease","volume":"63","author":"Krasnianski","year":"2006"},{"key":"b27-focus15328","series-title":"Brain","first-page":"2288","article-title":"Clinical findings and diagnostic tests in the MV2 subtype of sporadic CJD","volume":"129","author":"Krasnianski","year":"2006"},{"key":"b28-focus15328","series-title":"AJNR Am J Neuroradiol","first-page":"1945","article-title":"Cerebral white matter disruption in Creutzfeldt-Jakob disease","volume":"33","author":"Lee","year":"2012"},{"key":"b29-focus15328","series-title":"Science","first-page":"364","article-title":"Human prion protein cDNA: molecular cloning, chromosomal mapping, and biological implications","volume":"233","author":"Liao","year":"1986"},{"key":"b30-focus15328","series-title":"Lancet","first-page":"417","article-title":"Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion","volume":"363","author":"Llewelyn","year":"2004"},{"key":"b31-focus15328","series-title":"J Neurosci Rural Pract","first-page":"39","article-title":"A study of clinical profile, radiological and electroencephalographic characteristics of suspected Creutzfeldt-Jakob disease in a tertiary care centre in South India","volume":"6","author":"Mahale","year":"2015"},{"key":"b32-focus15328","series-title":"Emerg Infect Dis","first-page":"750","article-title":"Recent US Case of Variant Creutzfeldt-Jakob Disease-Global Implications","volume":"21","author":"Maheshwari","year":"2015"},{"key":"b33-focus15328","series-title":"Alzheimer Dis Assoc Disord","first-page":"46","article-title":"Creutzfeldt-Jakob disease: its origins","volume":"3","author":"Masters","year":"1989"},{"key":"b34-focus15328","series-title":"Ann Neurol","first-page":"278","article-title":"Real time quaking-induced conversion analysis of cerebrospinal fluid in sporadic Creutzfeldt-Jakob disease","volume":"72","author":"McGuire","year":"2012"},{"key":"b35-focus15328","series-title":"Eur J Hum Genet","first-page":"273","article-title":"Prion disease genetics","volume":"14","author":"Mead","year":"2006"},{"key":"b36-focus15328","series-title":"Neurology","first-page":"1544","article-title":"Sporadic Creutzfeldt-Jakob disease: clinical and diagnostic characteristics of the rare VV1 type","volume":"65","author":"Meissner","year":"2005"},{"key":"b37-focus15328","series-title":"Arch Neurol","first-page":"951","article-title":"Diffusion-weighted magnetic resonance imaging in probable Creutzfeldt-Jakob disease: a clinical-anatomic correlation","volume":"56","author":"Na","year":"1999"},{"key":"b38-focus15328","series-title":"Brain","first-page":"519","article-title":"Subacute spongiform encephalopathy\u2014a subacute form of encephalopathy attributable to vascular dysfunction (spongiform cerebral atrophy)","volume":"83","author":"Nevin","year":"1960"},{"key":"b39-focus15328","series-title":"N Engl J Med","first-page":"519","article-title":"A test for Creutzfeldt-Jakob disease using nasal brushings","volume":"371","author":"Orr\u00fa","year":"2014"},{"key":"b40-focus15328","series-title":"Neurology","first-page":"714","article-title":"Efficacy of flupirtine on cognitive function in patients with CJD: A double-blind study","volume":"62","author":"Otto","year":"2004"},{"key":"b41-focus15328","series-title":"Ann Neurol","first-page":"224","article-title":"Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects","volume":"46","author":"Parchi","year":"1999"},{"key":"b42-focus15328","series-title":"Folia Neuropathol","first-page":"20","article-title":"Molecular pathology, classification, and diagnosis of sporadic human prion disease variants","volume":"50","author":"Parchi","year":"2012"},{"key":"b43-focus15328","series-title":"Science","first-page":"136","article-title":"Novel proteinaceous infectious particles cause scrapie","volume":"216","author":"Prusiner","year":"1982"},{"key":"b44-focus15328","series-title":"Proc Natl Acad Sci U S A","first-page":"13363","article-title":"Prions","volume":"95","author":"Prusiner","year":"1998"},{"key":"b45-focus15328","series-title":"Proc Natl Acad Sci U S A","first-page":"3501","article-title":"Diagnosis of human prion disease","volume":"102","author":"Safar","year":"2005"},{"key":"b46-focus15328","series-title":"Acta Neuropathol","first-page":"327","article-title":"Brain biopsy in dementia: clinical indications and diagnostic approach","volume":"120","author":"Schott","year":"2010"},{"key":"b47-focus15328","series-title":"Neurology","first-page":"419","article-title":"Open biopsy in patients with acute progressive neurologic decline and absence of mass 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