Extensive cortical damage in a case of Creutzfeldt-Jacob disease: clinicoradiological correlations

M. Bergui1, G. B. Bradac1, G. Rossi2, L. Orsi3
1Neuroradiology, Ospedale S. G. Battista, University of Torino, Torino, Italy
2Neuropathology Department, Istituto Neurologico C. Besta, Milano, Italy
3Neurology, Ospedale S. G. Battista, University of Torino, Torino, Italy

Tóm tắt

MRI demonstrated extensive cortical involvement in a patient with pathologically proven Creutzfeldt-Jacob disease. The whole brain was atrophic; some of the supratentorial cortex, putamen and caudate nucleus gave high signal on T2-weighted images; the changes were more extensive on diffusion-weighted images (DWI). Comparison of the history, and the sites of atrophy and signal change suggested that the latter predominates in regions with long-lasting damage and prevalent gliosis, while high signal on DWI indicate current neuronal loss. This case widens the range of MRI findings in patients with Creutzfeldt-Jacob disease, and suggests that some information about the progression of the disease can be extracted from single MRI study.

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Tài liệu tham khảo

Parchi P, Castellani R, Capellari S, et al (1996) Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol 39: 767–778 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. Br Med J 312: 844 Grisoli M, Bruzzone M, Giaccone G, Tagliavini F, Savoiardo M, Bugiani 0 (1999) Value of MRI in diagnosis of Creutzfeldt-Jakob disease. J Neurol 246 [Suppl 1]: I34 Schroter A, Zerr I, Henkel K, Ischampa HI, Finkenstaedt M, Poser S (2000) Magnetic resonance imaging in the clinical diagnosis of Creutzfeldt-Jakob disease. Arch Neurol 57: 1751–1757 Zeidler M, Sellar RI, Collie DA, et al (2000) The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet 355: 1412–1418 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 Masters C, Richardson E (1978) Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease). The nature and progression of spongiform change. Brain 101: 333–344 Barboriak DP, Provenzale JM, Boyko OB (1994) MR diagnosis of Creutzfeldt-Jakob disease: significance of high signal intensity of the basal ganglia. Am J Roentgenol 162: 137–140 Finkenstaedt M, Szudra A, Zerr I, et al (1996) MR imaging of Creutzfeldt-Jakob disease. Radiology 199: 793–798 Na D, Suh C, Choi S, et al (1999) Diffusion-weighted magnetic resonance imaging in probable Creutzfeldt-Jakob disease. Arch Neurol 56: 951–957 Bahn MM, Parchi P (1999) Abnormal diffusion–weighted magnetic resonance images in Creutzfeldt-Jakob disease. Arch Neurol 56: 577–583 Demaerel P, Reiner L, Robberecht W, Sciot R, Wilms G (1999) Diffusion-weighted MIRI in sporadic Creutzfeldt-Jakob disease. Neurology 52: 205–208 Nitrini R, Mendonca RA, Huang N, LeBlanc A, Livramento JA, Marie SK (2001) Diffusion-weighted MRI in two cases of familial Creutzfeldt-Jakob disease. J Neurol Sci 184: 163–167 Yee AS, Simon JH, Anderson CA, Sze CI, Filley CJD (1999) Diffusion-weighted MRI of right hemisphere dysfunction in Creutzfeldt-Jakob disease. Neurology 52: 1514–1515 Schwaninger M, Winter R, Hacke W, et al (1997) Magnetic resonance imaging in Creutzfeldt-Jakob disease: evidence of focal involvement of the cortex. J Neurol Neurosurg Psychiatry 63: 408–409 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 23: 1164–1172 Tribl GG, Strasser G, Zeitlhofer J, et al (2002) Sequential MRI in a case of Creutzfeldt-Jakob disease. Neuroradiology 44: 223–226 Chung YL, Williams A, Ritchie D, et al (1999) Conflicting MRI signals from gliosis and neuronal vacuolation in prion diseases. NeuroReport 26: 3471–3477 Iwasaki Y, Ikeda K, Tagaya N, Kinoshita M (1994) Magnetic resonance imaging and neuropathological findings in two patients with Creutzfeldt-Jakob disease. J Neurol Sci 126: 228–231 Uemura A, O'uchi T, Sakamoto T, Yashiro N (2002) High signal of the striatum in sporadic Creutzfeldt-Jakob disease: sequential change on T2–weighted MRI. Neuroradiology 44: 314–318 Bruzzone M, Canciani B, Farina L, et al (1999) Experimental prion protein disease: comparison of neuropathology with magnetic resonance imaging. J Neurol 246 [Suppl 1]: I34 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: I28–134 Mao-Draayer Y, Braff SP, Nagle KJ, Pendlebury W, Penar PL, Shapiro RE (2002) Emerging patterns of diffusion–weighted MR imaging in Creutzfeldt-Jakob disease: case report and review of the literature. AJNR 23: 550–556 Matoba M, Tonami LI, Miyaji H, Yokota H, Yamamoto I (2001) Creutzfeldt-Jakob disease: serial changes on diffusion-weighted MRI. J Comput Assist Tomogr 25: 274–277 Uchino A, Yoshinaga M, Shiokawa O, Hata H, Ohno M. (1991) Serial MR imaging in Creutzfeldt–Jakob disease. Neuroradiology 33: 364–367