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Radiological assessment of Creutzfeldt-Jakob disease

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Abstract

Creutzfeldt-Jakob disease is a rare fatal neurodegenerative disorder, characterized by rapidly progressive dementia and neurological signs. There is a need for early and accurate clinical diagnosis in order to exclude any treatable disorder. Additionally, it is of public interest to differentiate the sporadic form of the disease from the variant CJD type (vCJD), which is probably transmitted from cattle infected with bovine spongiform encephalopathy (BSE). High signal in the striatum on T2-weighted, FLAIR and diffusion weighted (DW) MRI as well as cortical high signal in FLAIR and DW MRI are the classical findings in sCJD. The “pulvinar sign”, defined as high signal in the pulvinar thalami that is brighter than potential additional high signal in the basal ganglia, is considered pathognomonic for vCJD.

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Abbreviations

BSE:

bovine spongiform encephalopathy

DW:

diffusion weighted

EEG:

electroencephalogram

FFI:

fatal familial insomnia

FLAIR:

fluid attenuated inversion recovery

PD:

proton density

PSWCs:

periodic sharp wave complexes

sCJD:

sporadic Creutzfeldt-Jakob disease

SFI:

sporadic fatal insomnia

TSE:

transmissible spongiform encephalopathy

vCJD:

variant Creutzfeldt-Jakob disease

References

  1. Prusiner SB (1998) Prions. Proc Natl Acad Sci USA 95:13363–13383

    Article  PubMed  CAS  Google Scholar 

  2. Masters CL, Harris JO, Gajdusek DC, Gibbs CJ Jr, Bernoulli C, Asher DM (1979) Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann Neurol 5:177–188

    Article  PubMed  CAS  Google Scholar 

  3. World Health Organization (1998) Human transmissible spongiform encephalopathies. Weekly Epidemiological Record 73:361–365

    Google Scholar 

  4. Steinhoff BJ, Racker S, Herrendorf G, et al (1996) Accuracy and reliability of periodic sharp wave complexes in Creutzfeldt-Jakob disease. Arch Neurol 53:162–166

    PubMed  CAS  Google Scholar 

  5. Zerr I, Pocchiari M, Collins S et al (2000) Analysis of EEG and CSF 14-3-3 proteins as aids to the diagnosis of Creutzfeldt-Jakob disease. Neurology 55:811–815

    PubMed  CAS  Google Scholar 

  6. Steinhoff BJ, Zerr I, Glatting M, Schulz-Schaeffer W, Poser S, Kretzschmar HA (2004) Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease. Ann Neurol 56:702–708

    Article  PubMed  Google Scholar 

  7. Poser S, Mollenhauer B, Kraubeta A et al (1999) How to improve the clinical diagnosis of Creutzfeldt-Jakob disease. Brain 122 (Pt 12):2345–2351

    Article  PubMed  Google Scholar 

  8. Parchi P, Giese A, Capellari S et al (1999) Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 46:224–233

    Article  PubMed  CAS  Google Scholar 

  9. Tschampa HJ, Neumann M, Zerr I et al (2001) Patients with Alzheimer’s disease and dementia with Lewy bodies mistaken for Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 71:33–39

    Article  PubMed  CAS  Google Scholar 

  10. Boesenberg C, Schulz-Schaeffer WJ, Meissner B et al (2005) Clinical course in young patients with sporadic Creutzfeldt-Jakob disease. Ann Neurol 58(4):533–543

    Article  PubMed  Google Scholar 

  11. Windl O, Giese A, Schulz-Schaeffer W et al (1999) Molecular genetics of human prion diseases in Germany. Hum Genet 105:244–252

    Article  PubMed  CAS  Google Scholar 

  12. Kovacs GG, Puopolo M, Ladogana A et al (2005) Genetic prion disease: the EUROCJD experience. Hum Genet 1–9

  13. Shimizu S, Hoshi K, Muramoto T et al (1999) Creutzfeldt-Jakob disease with florid-type plaques after cadaveric dura mater grafting. Arch Neurol 56:357–362

    Article  PubMed  CAS  Google Scholar 

  14. Will RG, Ironside JW, Zeidler M et al (1996) A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 347:921–925

    Article  PubMed  CAS  Google Scholar 

  15. From the Centers for Disease Control and Prevention (1996) World Health Organization Consultation on public health issues related to bovine spongiform encephalopathy and the emergence of a new variant of Creutzfeldt-Jakob disease. JAMA 275:1305–1306

    Article  Google Scholar 

  16. Hill AF, Desbruslais M, Joiner S et al (1997) The same prion strain causes vCJD and BSE. Nature 389:448–50

    Article  PubMed  CAS  Google Scholar 

  17. Will RG, Zeidler M, Stewart GE et al (2000) Diagnosis of new variant Creutzfeldt-Jakob disease. Ann Neurol 47:575–582

    Article  PubMed  CAS  Google Scholar 

  18. Kretzschmar HA, Ironside JW, DeArmond SJ, Tateishi J (1996) Diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Arch Neurol 53:913–920

    PubMed  CAS  Google Scholar 

  19. Kropp S, Finkenstaedt M, Zerr I, Schroter A, Poser S (2000) Diffusion-weighted MRI in patients with Creutzfeldt-Jakob disease. Nervenarzt 71:91–95

    Article  PubMed  CAS  Google Scholar 

  20. Shiga Y, Miyazawa K, Sato S et al (2004) Diffusion-weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt-Jakob disease. Neurology 63:443–449

    PubMed  CAS  Google Scholar 

  21. Young GS, Geschwind MD, Fischbein NJ et al (2005) Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosis. AJNR Am J Neuroradiol 26:1551–1562

    PubMed  Google Scholar 

  22. Matoba M, Tonami H, Miyaji H, Yokota H, Yamamoto I (2001) Creutzfeldt-Jakob disease: serial changes on diffusion-weighted MRI. J Comput Assist Tomogr 25:274–277

    Article  PubMed  CAS  Google Scholar 

  23. Zeidler M, Collie DA, Macleod MA, Sellar RJ, Knight R (2001) FLAIR MRI in sporadic Creutzfeldt-Jakob disease. Neurology 56:282

    PubMed  CAS  Google Scholar 

  24. Gertz HJ, Henkes H, Cervos-Navarro J (1988) Creutzfeldt-Jakob disease: correlation of MRI and neuropathologic findings. Neurology 38:1481–1482

    PubMed  CAS  Google Scholar 

  25. Finkenstaedt M, Szudra A, Zerr I et al (1996) MR imaging of Creutzfeldt-Jakob disease. Radiology 199:793–798

    PubMed  CAS  Google Scholar 

  26. Barboriak DP, Provenzale JM, Boyko OB (1994) MR diagnosis of Creutzfeldt-Jakob disease: significance of high signal intensity of the basal ganglia. AJR Am J Roentgenol 162:137–140

    PubMed  CAS  Google Scholar 

  27. Milton WJ, Atlas SW, Lavi E, Mollman JE (1991) Magnetic resonance imaging of Creutzfeldt-Jacob disease. Ann Neurol 29:438–440

    Article  PubMed  CAS  Google Scholar 

  28. Tartaro A, Fulgente T, Delli PC, Bonomo L, Bocola V, Onofrj M (1993) MRI alterations as an early finding in Creutzfeld-Jakob disease. Eur J Radiol 17:155–158

    Article  PubMed  CAS  Google Scholar 

  29. Rother J, Schwartz A, Harle M, Wentz KU, Berlit P, Hennerici M (1992) Magnetic resonance imaging follow-up in Creutzfeldt-Jakob disease. J Neurol 239:404–406

    PubMed  CAS  Google Scholar 

  30. Pearl GS, Anderson RE (1989) Creutzfeldt-Jakob disease: high caudate signal on magnetic resonance imaging. South Med J 82:1177–1180

    PubMed  CAS  Google Scholar 

  31. Schroter A, Zerr I, Henkel K, Tschampa HJ, Finkenstaedt M, Poser S (2000) Magnetic resonance imaging in the clinical diagnosis of Creutzfeldt-Jakob disease. Arch Neurol 57:1751–1757

    Article  PubMed  CAS  Google Scholar 

  32. Tschampa HJ, Kallenberg K, Urbach H et al (2005) MRI in the diagnosis of sporadic Creutzfeldt-Jakob disease: a study on inter-observer agreement. Brain 128:2026–2033

    Article  PubMed  Google Scholar 

  33. de Priester JA, Jansen GH, de Kruijk JR, Wilmink JT (1999) New MRI findings in Creutzfeldt-Jakob disease: high signal in the globus pallidus on T1-weighted images. Neuroradiology 41:265–268

    Article  PubMed  Google Scholar 

  34. Kovanen J, Erkinjuntti T, Iivanainen M et al (1985) Cerebral MR and CT imaging in Creutzfeldt-Jakob disease. J Comput Assist Tomogr 9:125–128

    Article  PubMed  CAS  Google Scholar 

  35. Ukisu R, Kushihashi T, Kitanosono T et al (2005) Serial diffusion-weighted MRI of Creutzfeldt-Jakob disease. AJR Am J Roentgenol 184:560–566

    PubMed  Google Scholar 

  36. Bahn MM, Kido DK, Lin W, Pearlman AL (1997) Brain magnetic resonance diffusion abnormalities in Creutzfeldt-Jakob disease. Arch Neurol 54:1411–1415

    PubMed  CAS  Google Scholar 

  37. Bahn MM, Parchi P (1999) Abnormal diffusion-weighted magnetic resonance images in Creutzfeldt-Jakob disease. Arch Neurol 56:577–583

    Article  PubMed  CAS  Google Scholar 

  38. Yee AS, Simon JH, Anderson CA, Sze CI, Filley CM (1999) Diffusion-weighted MRI of right-hemisphere dysfunction in Creutzfeldt-Jakob disease. Neurology 52:1514–1515

    PubMed  CAS  Google Scholar 

  39. Demaerel P, Baert AL, Vanopdenbosch L, Robberecht W, Dom R (1997) Diffusion-weighted magnetic resonance imaging in Creutzfeldt-Jakob disease. Lancet 349:847–848

    Article  PubMed  CAS  Google Scholar 

  40. Demaerel P, Sciot R, Robberecht W et al (2003) Accuracy of diffusion-weighted MR imaging in the diagnosis of sporadic Creutzfeldt-Jakob disease. J Neurol 250:222–225

    Article  PubMed  Google Scholar 

  41. Murata T, Shiga Y, Higano S, Takahashi S, Mugikura S (2002) Conspicuity and evolution of lesions in creutzfeldt-jakob disease at diffusion-weighted imaging. AJNR Am J Neuroradiol 23:1164–1172

    PubMed  Google Scholar 

  42. Tschampa HJ, Murtz P, Flacke S, Paus S, Schild HH, Urbach H (2003) Thalamic involvement in sporadic Creutzfeldt-Jakob disease: a diffusion-weighted MR imaging study. AJNR Am J Neuroradiol 24:908–915

    PubMed  Google Scholar 

  43. Young GS, Lin Y, Chen N, Dillon WP, Wong S (2005) Abnormality of Apparent Diffusion Coefficient (ADC) in Somatosensory Cortex in Jakob-Creutzfeldt disease (CJD): Is Rolandic Cortex Really Spared? RSNA Scientific Assembly and Annual Meeting Program 517; SSQ12–08–517

    Google Scholar 

  44. Sellars RJ, Collie DA, Will RJ (2002) Progress in understanding Creutzfeldt-Jakob disease. AJNR Am J Neuroradiol 23:1070–1072

    PubMed  Google Scholar 

  45. Rowley HA, McCue J, Salamat MS, Fleming J (2004) Diffusion and Perfusion MRI in Creutzfeldt-Jakob disease. Proc Intl Soc Magn Reson Med 11:1340

    Google Scholar 

  46. Burdette JH, Durden DD, Elster AD, Yen YF (2001) High b-value diffusion-weighted MRI of normal brain. J Comput Assist Tomogr 25:515–519

    Article  PubMed  CAS  Google Scholar 

  47. Tschampa HJ, Kallenberg K, Zerr I, Kretzschmar HA, Knauth M, Urbach H (2005) Pattern of cortical changes in sporadic Creutzfeldt-Jakob disease. RSNA Scientific Assembly and Annual Meeting Program, 515

  48. Poon MA, Stuckey S, Storey E (2001) MRI evidence of cerebellar and hippocampal involvement in Creutzfeldt-Jakob disease. Neuroradiology 43:746–749

    Article  PubMed  CAS  Google Scholar 

  49. Tribl GG, Strasser G, Zeitlhofer J et al (2002) Sequential MRI in a case of Creutzfeldt-Jakob disease. Neuroradiology 44:223–226

    Article  PubMed  CAS  Google Scholar 

  50. Matsusue E, Kinoshita T, Sugihara S, Fujii S, Ogawa T, Ohama E (2004) White matter lesions in panencephalopathic type of Creutzfeldt-Jakob disease: MR imaging and pathologic correlations. AJNR Am J Neuroradiol 25:910–918

    PubMed  Google Scholar 

  51. Zeidler M, Will RG, Ironside JW, Sellar R, Wardlaw J (1996) Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. Magnetic resonance imaging is not a sensitive test for Creutzfeldt-Jakob disease. BMJ 312:844

    PubMed  CAS  Google Scholar 

  52. Hirai T, Korogi Y, Yoshizumi K, Shigematsu Y, Sugahara T, Takahashi M (2000) Limbic lobe of the human brain: evaluation with turbo fluid-attenuated inversion-recovery MR imaging. Radiology 215:470–475

    PubMed  CAS  Google Scholar 

  53. Collie DA, Summers DM, Sellar RJ et al (2003) Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases. AJNR Am J Neuroradiol 24:1560–1569

    PubMed  Google Scholar 

  54. Zeidler M, Sellar RJ, Collie DA et al (2000) The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet 355:1412–1418

    Article  PubMed  CAS  Google Scholar 

  55. Martindale J, Geschwind MD, De Armond S et al (2003) Sporadic Creutzfeldt-Jakob disease mimicking variant Creutzfeldt-Jakob disease. Arch Neurol 60:767–770

    Article  PubMed  Google Scholar 

  56. Rabinstein AA, Whiteman ML, Shebert RT (2002) Abnormal diffusion-weighted magnetic resonance imaging in Creutzfeldt-Jakob disease following corneal transplantations. Arch Neurol 59:637–639

    Article  PubMed  Google Scholar 

  57. Meissner B, Kortner K, Bartl M et al (2004) Sporadic Creutzfeldt-Jakob disease: magnetic resonance imaging and clinical findings. Neurology 63:450–456

    PubMed  CAS  Google Scholar 

  58. Kropp S, Schulz-Schaeffer WJ, Finkenstaedt M et al (1999) The Heidenhain variant of Creutzfeldt-Jakob disease. Arch Neurol 56:55–61

    Article  PubMed  CAS  Google Scholar 

  59. Na DL, Suh CK, Choi SH et al (1999) Diffusion-weighted magnetic resonance imaging in probable Creutzfeldt-Jakob disease: a clinical-anatomic correlation. Arch Neurol 56:951–957

    Article  PubMed  CAS  Google Scholar 

  60. Chung YL, Williams A, Ritchie D et al (1999) Conflicting MRI signals from gliosis and neuronal vacuolation in prion diseases. NeuroReport 10:3471–3477

    Article  PubMed  CAS  Google Scholar 

  61. Mittal S, Farmer P, Kalina P, Kingsley PB, Halperin J (2002) Correlation of diffusion-weighted magnetic resonance imaging with neuropathology in Creutzfeldt-Jakob disease. Arch Neurol 59:128–134

    Article  PubMed  Google Scholar 

  62. Urbach H, Klisch J, Wolf HK, Brechtelsbauer D, Gass S, Solymosi L (1998) MRI in sporadic Creutzfeldt-Jakob disease: correlation with clinical and neuropathological data. Neuroradiology 40:65–70

    Article  PubMed  CAS  Google Scholar 

  63. Safar JG, Geschwind MD, Deering C et al (2005) Diagnosis of human prion disease. Proc Natl Acad Sci USA 102:3501–3506

    Article  PubMed  CAS  Google Scholar 

  64. Haik S, Dormont D, Faucheux BA, Marsault C, Hauw JJ (2002) Prion protein deposits match magnetic resonance imaging signal abnormalities in Creutzfeldt-Jakob disease. Ann Neurol 51:797–799

    Article  PubMed  Google Scholar 

  65. Russmann H, Vingerhoets F, Miklossy J et al (2005) Sporadic Creutzfeldt-Jakob disease: a comparison of pathological findings and diffusion weighted imaging. J Neurol 252:338–342

    Article  PubMed  Google Scholar 

  66. Henkel K, Zerr I, Hertel A et al (2002) Positron emission tomography with [(18)F]FDG in the diagnosis of Creutzfeldt-Jakob disease (CJD). J Neurol 249:699–705

    Article  PubMed  Google Scholar 

  67. de Silva R, Patterson J, Hadley D, Russell A, Turner M, Zeidler M (1998) Single photon emission computed tomography in the identification of new variant Creutzfeldt-Jakob disease: case reports. BMJ 316:593–594

    PubMed  Google Scholar 

  68. Collie DA, Sellar RJ, Zeidler M, Colchester AC, Knight R, Will RG (2001) MRI of Creutzfeldt-Jakob disease: imaging features and recommended MRI protocol. Clin Radiol 56:726–739

    Article  PubMed  CAS  Google Scholar 

  69. Zerr I, Schulz-Schaeffer WJ, Giese A et al (2000) Current clinical diagnosis in Creutzfeldt-Jakob disease: identification of uncommon variants. Ann Neurol 48:323–329

    Article  PubMed  CAS  Google Scholar 

  70. Castellani RJ, Colucci M, Xie Z et al (2004) Sensitivity of 14-3-3 protein test varies in subtypes of sporadic Creutzfeldt-Jakob disease. Neurology 63:436–442

    PubMed  CAS  Google Scholar 

  71. Hamaguchi T, Kitamoto T, Sato T et al (2005) Clinical diagnosis of MM2-type sporadic Creutzfeldt-Jakob disease. Neurology 64:643–648

    PubMed  CAS  Google Scholar 

  72. Parchi P, Capellari S, Chin S et al (1999) A subtype of sporadic prion disease mimicking fatal familial insomnia. Neurology 52:1757–1763

    PubMed  CAS  Google Scholar 

  73. Samman I, Schulz-Schaeffer WJ, Wohrle JC, Sommer A, Kretzschmar HA, Hennerici M (1999) Clinical range and MRI in Creutzfeldt-Jakob disease with heterozygosity at codon 129 and prion protein type 2. J Neurol Neurosurg Psychiatry 67:678–681

    Article  PubMed  CAS  Google Scholar 

  74. Fukushima R, Shiga Y, Nakamura M, Fujimori J, Kitamoto T, Yoshida Y (2004) MRI characteristics of sporadic CJD with valine homozygosity at codon 129 of the prion protein gene and PrPSc type 2 in Japan. J Neurol Neurosurg Psychiatry 75:485–487

    Article  PubMed  CAS  Google Scholar 

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Acknowledgements

We thank Hanno Schimikowski for his help with figure editing and Carsten Krautmacher for critical reading of the manuscript.

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Authors and Affiliations

  1. Department of Radiology, University of Bonn, Sigmund-Freud-Strasse 25, 53105, Bonn, Germany

    Henriette J. Tschampa & Horst Urbach

  2. National Reference Center for TSE Surveillance at the Department of Neurology, University of Göttingen, Göttingen, Germany

    Inga Zerr

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  1. Henriette J. Tschampa
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  2. Inga Zerr
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  3. Horst Urbach
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Corresponding author

Correspondence to Henriette J. Tschampa.

Additional information

This article gives an overview on imaging features of sporadic and variant CJD. It discusses the clinical presentation and pathology of CJD, as well as the differential diagnoses on clinical and MR grounds.

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Tschampa, H.J., Zerr, I. & Urbach, H. Radiological assessment of Creutzfeldt-Jakob disease. Eur Radiol 17, 1200–1211 (2007). https://doi.org/10.1007/s00330-006-0456-2

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