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Creutzfeldt-Jakob Disease (CJD)
Santhosh Gaddikeri, MD; Miral D. Jhaveri, MD, MBA
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Creutzfeldt-Jakob disease (CJD)
            • Sporadic Creutzfeldt-Jakob disease (sCJD)
            • Variant Creutzfeldt-Jakob disease (vCJD)
          • Definitions

            • Rapidly progressing, fatal, neurodegenerative disorder caused by prion (proteinaceous infectious particle devoid of DNA and RNA)
              • Transmissible spongiform encephalopathy

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Nuclear Medicine Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Staging, Grading, & Classification

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Alaoui A et al: [MRI role in Creutzfeldt-Jakob disease: about a case.] Pan Afr Med J. 32:95, 2019
                                          2. Baldwin KJ et al: Prion disease. Semin Neurol. 39(4):428-39, 2019
                                          3. Groveman BR et al: Sporadic Creutzfeldt-Jakob disease prion infection of human cerebral organoids. Acta Neuropathol Commun. 7(1):12, 2019
                                          4. Hayashi Y et al: Clinicopathological findings of an MM2-cortical-type sporadic Creutzfeldt-Jakob disease patient with cortical blindness during a course of glaucoma and age-related macular degeneration. Prion. 13(1):124-31, 2019
                                          5. Llorens F et al: Plasma total prion protein as a potential biomarker for neurodegenerative dementia: diagnostic accuracy in the spectrum of prion diseases. Neuropathol Appl Neurobiol. ePub, 2019
                                          6. Muniz BC et al: The Heidenhain variant of Creutzfeldt-Jakob disease. Radiol Bras. 52(3):199-200, 2019
                                          7. Xu Y et al: Sporadic Creutzfeldt-Jakob disease presenting as dizziness and cognitive decline: a case report. Medicine (Baltimore). 98(24):e16002, 2019
                                          8. Fragoso DC et al: Imaging of Creutzfeldt-Jakob disease: imaging patterns and their differential diagnosis. Radiographics. 37(1):234-57, 2017
                                          9. Koeller KK et al: Viral and prion infections of the central nervous system: radiologic-pathologic correlation: from the radiologic pathology archives. Radiographics. 37(1):199-233, 2017
                                          10. Caobelli F et al: The role of neuroimaging in evaluating patients affected by Creutzfeldt-Jakob disease: a systematic review of the literature. J Neuroimaging. 25(1):2-13, 2015
                                          11. Kim MO et al: Clinical update of Jakob-Creutzfeldt disease. Curr Opin Neurol. 28(3):302-10, 2015
                                          12. Felix-Morais R et al: Creutzfeldt-Jakob disease: typical imaging findings. BMJ Case Rep. 2014, 2014
                                          13. Risacher SL et al: Neuroimaging biomarkers of neurodegenerative diseases and dementia. Semin Neurol. 33(4):386-416, 2013
                                          14. Appleby BS et al: Characteristics of established and proposed sporadic Creutzfeldt-Jakob disease variants. Arch Neurol. 66(2):208-15, 2009
                                          15. Iwasaki Y et al: Clinical diagnosis of Creutzfeldt-Jakob disease: accuracy based on analysis of autopsy-confirmed cases. J Neurol Sci. 277(1-2):119-23, 2009
                                          16. Josephs KA et al: Rapidly progressive neurodegenerative dementias. Arch Neurol. 66(2):201-7, 2009
                                          17. Manners DN et al: Pathologic correlates of diffusion MRI changes in Creutzfeldt-Jakob disease. Neurology. 72(16):1425-31, 2009
                                          18. Meissner B et al: MRI lesion profiles in sporadic Creutzfeldt-Jakob disease. Neurology. 72(23):1994-2001, 2009
                                          19. Clarençon F et al: MRI and FDG PET/CT findings in a case of probable Heidenhain variant Creutzfeldt-Jakob disease. J Neuroradiol. 35(4):240-3, 2008
                                          20. Fulbright RK et al: MR imaging of familial Creutzfeldt-Jakob disease: a blinded and controlled study. AJNR Am J Neuroradiol. 29(9):1638-43, 2008
                                          21. Heinemann U et al: Brain biopsy in patients with suspected Creutzfeldt-Jakob disease. J Neurosurg. 109(4):735-41, 2008
                                          22. Meissner B et al: Isolated cortical signal increase on MR imaging as a frequent lesion pattern in sporadic Creutzfeldt-Jakob disease. AJNR Am J Neuroradiol. 29(8):1519-24, 2008
                                          23. Ward HJ et al: Risk factors for sporadic Creutzfeldt-Jakob disease. Ann Neurol. 63(3):347-54, 2008
                                          24. Yi SH et al: Relationship between clinical course and diffusion-weighted MRI findings in sporadic Creutzfeldt-Jakob Disease. Neurol Sci. 29(4):251-5, 2008
                                          25. Kallenberg K et al: Creutzfeldt-Jakob disease: comparative analysis of MR imaging sequences. AJNR Am J Neuroradiol. 27(7):1459-62, 2006
                                          26. Lin YR et al: Creutzfeldt-jakob disease involvement of rolandic cortex: a quantitative apparent diffusion coefficient evaluation. AJNR Am J Neuroradiol. 27(8):1755-9, 2006
                                          27. Tschampa HJ et al: MRI in the diagnosis of sporadic Creutzfeldt-Jakob disease: a study on inter-observer agreement. Brain. 128(Pt 9):2026-33, 2005
                                          28. Young GS et al: Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosis. AJNR Am J Neuroradiol. 26(6):1551-62, 2005
                                          29. Collins SJ et al: Transmissible spongiform encephalopathies. Lancet. 363(9402):51-61, 2004
                                          30. Summers DM et al: The pulvinar sign in variant Creutzfeldt-Jakob disease. Arch Neurol. 61(3):446-7, 2004
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Clinical Issues

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Creutzfeldt-Jakob disease (CJD)
                                                    • Sporadic Creutzfeldt-Jakob disease (sCJD)
                                                    • Variant Creutzfeldt-Jakob disease (vCJD)
                                                  • Definitions

                                                    • Rapidly progressing, fatal, neurodegenerative disorder caused by prion (proteinaceous infectious particle devoid of DNA and RNA)
                                                      • Transmissible spongiform encephalopathy

                                                  IMAGING

                                                  • General Features

                                                    • CT Findings

                                                      • MR Findings

                                                        • Nuclear Medicine Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Staging, Grading, & Classification

                                                                  • Gross Pathologic & Surgical Features

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              DIAGNOSTIC CHECKLIST

                                                                              • Consider

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Alaoui A et al: [MRI role in Creutzfeldt-Jakob disease: about a case.] Pan Afr Med J. 32:95, 2019
                                                                                  2. Baldwin KJ et al: Prion disease. Semin Neurol. 39(4):428-39, 2019
                                                                                  3. Groveman BR et al: Sporadic Creutzfeldt-Jakob disease prion infection of human cerebral organoids. Acta Neuropathol Commun. 7(1):12, 2019
                                                                                  4. Hayashi Y et al: Clinicopathological findings of an MM2-cortical-type sporadic Creutzfeldt-Jakob disease patient with cortical blindness during a course of glaucoma and age-related macular degeneration. Prion. 13(1):124-31, 2019
                                                                                  5. Llorens F et al: Plasma total prion protein as a potential biomarker for neurodegenerative dementia: diagnostic accuracy in the spectrum of prion diseases. Neuropathol Appl Neurobiol. ePub, 2019
                                                                                  6. Muniz BC et al: The Heidenhain variant of Creutzfeldt-Jakob disease. Radiol Bras. 52(3):199-200, 2019
                                                                                  7. Xu Y et al: Sporadic Creutzfeldt-Jakob disease presenting as dizziness and cognitive decline: a case report. Medicine (Baltimore). 98(24):e16002, 2019
                                                                                  8. Fragoso DC et al: Imaging of Creutzfeldt-Jakob disease: imaging patterns and their differential diagnosis. Radiographics. 37(1):234-57, 2017
                                                                                  9. Koeller KK et al: Viral and prion infections of the central nervous system: radiologic-pathologic correlation: from the radiologic pathology archives. Radiographics. 37(1):199-233, 2017
                                                                                  10. Caobelli F et al: The role of neuroimaging in evaluating patients affected by Creutzfeldt-Jakob disease: a systematic review of the literature. J Neuroimaging. 25(1):2-13, 2015
                                                                                  11. Kim MO et al: Clinical update of Jakob-Creutzfeldt disease. Curr Opin Neurol. 28(3):302-10, 2015
                                                                                  12. Felix-Morais R et al: Creutzfeldt-Jakob disease: typical imaging findings. BMJ Case Rep. 2014, 2014
                                                                                  13. Risacher SL et al: Neuroimaging biomarkers of neurodegenerative diseases and dementia. Semin Neurol. 33(4):386-416, 2013
                                                                                  14. Appleby BS et al: Characteristics of established and proposed sporadic Creutzfeldt-Jakob disease variants. Arch Neurol. 66(2):208-15, 2009
                                                                                  15. Iwasaki Y et al: Clinical diagnosis of Creutzfeldt-Jakob disease: accuracy based on analysis of autopsy-confirmed cases. J Neurol Sci. 277(1-2):119-23, 2009
                                                                                  16. Josephs KA et al: Rapidly progressive neurodegenerative dementias. Arch Neurol. 66(2):201-7, 2009
                                                                                  17. Manners DN et al: Pathologic correlates of diffusion MRI changes in Creutzfeldt-Jakob disease. Neurology. 72(16):1425-31, 2009
                                                                                  18. Meissner B et al: MRI lesion profiles in sporadic Creutzfeldt-Jakob disease. Neurology. 72(23):1994-2001, 2009
                                                                                  19. Clarençon F et al: MRI and FDG PET/CT findings in a case of probable Heidenhain variant Creutzfeldt-Jakob disease. J Neuroradiol. 35(4):240-3, 2008
                                                                                  20. Fulbright RK et al: MR imaging of familial Creutzfeldt-Jakob disease: a blinded and controlled study. AJNR Am J Neuroradiol. 29(9):1638-43, 2008
                                                                                  21. Heinemann U et al: Brain biopsy in patients with suspected Creutzfeldt-Jakob disease. J Neurosurg. 109(4):735-41, 2008
                                                                                  22. Meissner B et al: Isolated cortical signal increase on MR imaging as a frequent lesion pattern in sporadic Creutzfeldt-Jakob disease. AJNR Am J Neuroradiol. 29(8):1519-24, 2008
                                                                                  23. Ward HJ et al: Risk factors for sporadic Creutzfeldt-Jakob disease. Ann Neurol. 63(3):347-54, 2008
                                                                                  24. Yi SH et al: Relationship between clinical course and diffusion-weighted MRI findings in sporadic Creutzfeldt-Jakob Disease. Neurol Sci. 29(4):251-5, 2008
                                                                                  25. Kallenberg K et al: Creutzfeldt-Jakob disease: comparative analysis of MR imaging sequences. AJNR Am J Neuroradiol. 27(7):1459-62, 2006
                                                                                  26. Lin YR et al: Creutzfeldt-jakob disease involvement of rolandic cortex: a quantitative apparent diffusion coefficient evaluation. AJNR Am J Neuroradiol. 27(8):1755-9, 2006
                                                                                  27. Tschampa HJ et al: MRI in the diagnosis of sporadic Creutzfeldt-Jakob disease: a study on inter-observer agreement. Brain. 128(Pt 9):2026-33, 2005
                                                                                  28. Young GS et al: Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosis. AJNR Am J Neuroradiol. 26(6):1551-62, 2005
                                                                                  29. Collins SJ et al: Transmissible spongiform encephalopathies. Lancet. 363(9402):51-61, 2004
                                                                                  30. Summers DM et al: The pulvinar sign in variant Creutzfeldt-Jakob disease. Arch Neurol. 61(3):446-7, 2004