Research ArticleBRAIN
MR Imaging of Familial Creutzfeldt-Jakob Disease: A Blinded and Controlled Study
R.K. Fulbright, C. Hoffmann, H. Lee, A. Pozamantir, J. Chapman and I. Prohovnik
American Journal of Neuroradiology October 2008, 29 (9) 1638-1643; DOI: https://doi.org/10.3174/ajnr.A1217
R.K. Fulbright
C. Hoffmann
H. Lee
A. Pozamantir
J. Chapman

References
- ↵Johnson RT, Gibbs CJ Jr. Creutzfeldt-Jakob disease and related transmissible spongiform encephalopathies. N Engl J Med 1998;339:1994–2004
- ↵Chapman J, Ben-Israel J, Goldhammer Y, et al. The risk of developing Creutzfeldt-Jakob disease in subjects with the PRNP gene codon 200 point mutation. Neurology 1994;44:1683–86
- ↵Bahn MM, Parchi P. Abnormal diffusion-weighted magnetic resonance images in Creutzfeldt-Jakob disease. Arch Neurol 1999;56:577–83
- ↵Demaerel P, Baert AL, Vanopdenbosch L, et al. Diffusion-weighted magnetic resonance imaging in Creutzfeldt-Jakob disease. Lancet 1997;349:847–48
- ↵Finkenstaedt M, Szudra A, Zerr I, et al. MR imaging of Creutzfeldt-Jakob disease. Radiology 1996;199:793–98
- Mao-Draayer Y, Braff SP, Nagle KJ, et al. Emerging patterns of diffusion-weighted MR imaging in Creutzfeldt-Jakob disease: case report and review of the literature. AJNR Am J Neuroradiol 2002;23:550–56
- ↵Murata T, Shiga Y, Higano S, et al. Conspicuity and evolution of lesions in Creutzfeldt-Jakob disease at diffusion-weighted imaging. AJNR Am J Neuroradiol 2002;23:1164–72
- Na DL, Suh CK, Choi SH, et al. Diffusion-weighted magnetic resonance imaging in probable Creutzfeldt-Jakob disease: a clinical-anatomic correlation. Arch Neurol 1999;56:951–57
- Samman I, Schulz-Schaeffer WJ, Wohrle JC, et al. Clinical range and MRI in Creutzfeldt-Jakob disease with heterozygosity at codon 129 and prion protein type 2. J Neurol Neurosurg Psychiatry 1999;67:678–81
- ↵Ukisu R, Kushihashi T, Kitanosono T, et al. Serial diffusion-weighted MRI of Creutzfeldt-Jakob disease. AJR Am J Roentgenol 2005;184:560–66
- ↵Yee AS, Simon JH, Anderson CA, et al. Diffusion-weighted MRI of right-hemisphere dysfunction in Creutzfeldt-Jakob disease. Neurology 1999;52:1514–15
- ↵Yoon SS, Chan S, Chin S, et al. MRI of Creutzfeldt-Jakob disease: asymmetric high signal intensity of the basal ganglia. Neurology 1995;45:1932–33
- ↵Gambetti P, Kong Q, Zou W, et al. Sporadic and familial CJD: classification and characterisation. Br Med Bull 2003;66:213–39
- ↵Ishida S, Sugino M, Koizumi N, et al. Serial MRI in early Creutzfeldt-Jacob disease with a point mutation of prion protein at codon 180. Neuroradiology 1995;37:531–34
- ↵Mittal S, Farmer P, Kalina P, et al. Correlation of diffusion-weighted magnetic resonance imaging with neuropathology in Creutzfeldt-Jakob disease. Arch Neurol 2002;59:128–34
- ↵
- Satoh A, Goto H, Satoh H, et al. A case of Creutzfeldt-Jakob disease with a point mutation at codon 232: correlation of MRI and neurologic findings. Neurology 1997;49:1469–70
- ↵
- ↵Lin YR, Young GS, Chen NK, et al. Creutzfeldt-Jakob disease involvement of rolandic cortex: a quantitative apparent diffusion coefficient evaluation. AJNR Am J Neuroradiol 2006;27:1755–59
- ↵Shiga Y, Miyazawa K, Sato S, et al. Diffusion-weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt-Jakob disease. Neurology 2004;63:443–49
- ↵Young GS, Geschwind MD, Fischbein NJ, et al. Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosis. AJNR Am J Neuroradiol 2005;26:1551–62
- ↵Bergui M, Bradac GB, Rossi G, et al. Extensive cortical damage in a case of Creutzfeldt-Jacob disease: clinicoradiological correlations. Neuroradiology 2003;45:304–07
- ↵Goldfarb LG, Korczyn AD, Brown P, et al. Mutation in codon 200 of scrapie amyloid precursor gene linked to Creutzfeldt-Jakob disease in Sephardic Jews of Libyan and non-Libyan origin. Lancet 1990;336:637–38
- Hsiao K, Meiner Z, Kahana E, et al. Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease. N Engl J Med 1991;324:1091–97
- ↵Kahana E, Alter M, Braham J, et al. Creutzfeldt-Jakob disease: focus among Libyan Jews in Israel. Science 1974;183:90–91
- ↵Fulbright RK, Kingsley PB, Guo X, et al. The imaging appearance of Creutzfeldt-Jakob disease caused by the E200K mutation. Magn Reson Imaging 2006;24:1121–29
- ↵Satoh K, Shirabe S, Tsujino A, et al. Total tau protein in cerebrospinal fluid and diffusion-weighted MRI as an early diagnostic marker for Creutzfeldt-Jakob disease. Dement Geriatr Cogn Disord 2007;24:207–12
- ↵Goldgaber D, Goldfarb LG, Brown P, et al. Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-Straussler-Scheinker's syndrome. Exp Neurol 1989;106:204–06
- ↵Folstein MF, Folstein SE, McHugh PR. “Mini-mental state.” A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 1975;12:189–98
- ↵Chapman J, Cohen OS, Ephraty L, et al. The CJD rating scale: a new tool for evaluation of neurologic symptomatology in early CJD patients [abstract]. Presented at the 59th Annual Meeting of the American Academy of Neurology, April 28–May 5,2007; Boston, Mass;A140
- ↵
- ↵Kovacs GG, Puopolo M, Ladogana A, et al. Genetic prion disease: the EUROCJD experience. Hum Genet 2005;118:166–74
- ↵Tschampa HJ, Kallenberg K, Urbach H, et al. MRI in the diagnosis of sporadic Creutzfeldt-Jakob disease: a study on inter-observer agreement. Brain 2005;128:2026–33
- ↵Tschampa HJ, Zerr I, Urbach H. Radiological assessment of Creutzfeldt-Jakob disease. Eur Radiol 2007;17:1200–11
- ↵Zeidler M, Collie DA, Macleod MA, et al. FLAIR MRI in sporadic Creutzfeldt-Jakob disease. Neurology 2001;56:282
- ↵Kallenberg K, Schulz-Schaeffer WJ, Jastrow U, et al. Creutzfeldt-Jakob disease: comparative analysis of MR imaging sequences. AJNR Am J Neuroradiol 2006;27:1459–62
- ↵Meissner B, Kortner K, Bartl M, et al. Sporadic Creutzfeldt-Jakob disease: magnetic resonance imaging and clinical findings. Neurology 2004;63:450–56
- ↵Tschampa HJ, Kallenberg K, Kretzschmar HA, et al. Pattern of cortical changes in sporadic Creutzfeldt-Jakob disease. AJNR Am J Neuroradiol 2007;28:1114–18
- ↵Hirai T, Korogi Y, Yoshizumi K, et al. Limbic lobe of the human brain: evaluation with turbo fluid-attenuated inversion-recovery MR imaging. Radiology 2000;215:470–75
- ↵
- ↵Martindale J, Geschwind MD, De Armond S, et al. Sporadic Creutzfeldt-Jakob disease mimicking variant Creutzfeldt-Jakob disease. Arch Neurol 2003;60:767–70
- ↵Tschampa HJ, Murtz P, Flacke S, et al. Thalamic involvement in sporadic Creutzfeldt-Jakob disease: a diffusion-weighted MR imaging study. AJNR Am J Neuroradiol 2003;24:908–15
- ↵Haik S, Brandel JP, Oppenheim C, et al. Sporadic CJD clinically mimicking variant CJD with bilateral increased signal in the pulvinar. Neurology 2002;58:148–49
- ↵Urbach H, Klisch J, Wolf HK, et al. MRI in sporadic Creutzfeldt-Jakob disease: correlation with clinical and neuropathological data. Neuroradiology 1998;40:65–70
- ↵Haik S, Dormont D, Faucheux BA, et al. Prion protein deposits match magnetic resonance imaging signal abnormalities in Creutzfeldt-Jakob disease. Ann Neurol 2002;51:797–99
- ↵
- ↵Freeborough PA, Fox NC. Modeling brain deformations in Alzheimer disease by fluid registration of serial 3D MR images. J Comput Assist Tomogr 1998;22:838–43
- ↵Rene R, Campdelacreu J, Ferrer I, et al. Familial Creutzfeldt-Jakob disease with E200K mutation presenting with neurosensorial hypoacusis. J Neurol Neurosurg Psychiatry 2007;78:103–04
- ↵Hoffman CC, Fulbright RK, Konen E, et al. Early detection of familial Jakob-Creutzfeldt disease: comparison of T-2, FLAIR and DTI techniques. Presented at the Radiological Society of North America, December 1,2006; Chicago, Ill.
- ↵Cohen OS, Hoffman C, Chapman J, et al. Correlation between DTI and neurological symptoms in patients with familial Creutzfeldt-Jakob disease [abstract]. Presented at the 59th Annual Meeting of the American Academy of Neurology,2007; Boston, Mass;A140
- ↵Schroter A, Zerr I, Henkel K, et al. Magnetic resonance imaging in the clinical diagnosis of Creutzfeldt-Jakob disease. Arch Neurol 2000;57:1751–57
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MR Imaging of Familial Creutzfeldt-Jakob Disease: A Blinded and Controlled Study
R.K. Fulbright, C. Hoffmann, H. Lee, A. Pozamantir, J. Chapman, I. Prohovnik
American Journal of Neuroradiology Oct 2008, 29 (9) 1638-1643; DOI: 10.3174/ajnr.A1217
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