The Neuroradiology Journal 19: 297-300, 2006

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Creutzfeldt-Jakob Disease The Value of Diffusion-Weighted MRI S. PURKAYASTHA, A.K. GUPTA, N.K. BODHEY Department of Imaging Sciences & Interventional Radiology, Sree Chitra Tirunal Institute for Medical Sciences & Technology; Trivandrum, India

Key words: Creutzfeldt-Jakob disease, MRI, diffusion-weighted imaging.

SUMMARY – Creutzfeldt-Jakob disease (CJD) is a transmissible, progressive, fatal spongiform encephalopathy. An early and accurate diagnosis is important to prevent disease transmission, but diagnosis is not easy, especially in the early stage of the disease. MRI plays an important role in the early diagnosis and differentiating the entity form other disorders.

Introduction Creutzfeldt-Jakob disease (CJD) is a transmissible, progressive, fatal spongiform encephalopathy 1. The transmission of bovine spongiform encephalopathy to humans is described as variant CJD 2. The cardinal manifestations of the disease are rapidly progressive dementia, generalized myoclonus, and periodic sharp wave complexes (PSWCs) on EEG. However, cases that do not consistently show such typical manifestations have been recognized, and the spectrum of disease manifestations has been extending 3,4. An early and accurate diagnosis is important to prevent disease transmission, but diagnosis is not easy, especially in the early stage of the disease. MRI plays an important role in the early diagnosis and indifferentiating the entity form other disorders. Case report This 63-year-old male patient presented with gradual onset of dementia with rapid progression over 4-6 weeks. He had myoclonic jerks. On examination he had global mental dysfunction, vertical gaze restriction, extrapyramidal signs and myoclonic jerks. With a provisional diagnosis of CJD, the patient was subjected to MRI brain. It showed T2-hyperintense lesion bilateraly in corpus striatum and thalami (figure 1). Hyperintense areas are also seen in T1-

weighted images in globi pallidi (figure 2). The diffusion weighted MRI showed restricted diffusion in the above-mentioned regions (figure 3). Although bilateral T2- hyperintense lesions in corpus striatum carries few differential diagnosis, the restricted diffusion points towards the possibility of CJD. Discussion MRI had not been thought to be a sensitive noninvasive diagnostic test of CJD; it was previously thought that EEG was the most reliable diagnostic test 5,6. Increased signal intensity in the basal ganglia on T2 was first described in 1988, and it was demonstrated that MRI was useful in depicting the lesions of CJD 7,8. Diffusion-weighted images (DWI) is very useful to distinguish CJD from AD, vascular dementia, and dementia with Lewy bodies, which are the major differential diagnoses of CJD and account for the vast majority of dementia in elderly patients 9,10. DWI of mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), Wilson disease, and Wernicke encephalopathy can demonstrate similar abnormalities. However, the clinical course and laboratory findings easily distinguish them from CJD. DWI in hypoglycemia, anoxia, and reversible posterior leukoencephalopathy syndrome has also been reported to demonstrate high-intensity lesions similar to those of CJD 297

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Figure 1 A,B) Axial T2 weighted images and (C) Flair axial image show bilateral hyperintense signal in corpus striatum and posteromedial thalami.

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Figure 2 A) Axial T1 weighted image shows hyperintense signal in bilateral globus pallidi. B) Axial gradient image shows hypointense signal in the corresponding areas suggesting mineralization.

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The Neuroradiology Journal 19: 297-300, 2006

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Figure 3 A,B) Diffusion weighted images show restricted diffusion in corpus striatum and posteromedial thalami. C,D) Apparent Diffusion Coefficient (ADC) maps in the corresponding areas are hypointense.

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. However, these have peculiar episodes, and the onset is apparently different from that of CJD. The exact mechanism leading to the presence of hyperintense signals in the basal ganglia on MRI scans is yet unknown. A possible link with spongiform or gliotic changes in the brain tissue has been discussed in this context 8,14,15. It was recently reported that signal changes on MRI matched neuropathologically detectable prion protein deposits 16. Hyperintense signal on T1 weighted images in globi pallidi is seen in the present case. It shows hypointensity in gradient recalled image probably suggesting calcification or mineralization 11,12,13

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in these specific areas. Recently Young et al. evaluated the abnormalities on DWI and fluidattenuated inversion recovery (FLAIR) in CJD and found that specific patterns of abnormality on DWI and FLAIR images are highly sensitive and specific for CJD; these sequences should be performed whenever CJD is suspected 17. So, we feel that conventional MRI along with DWI can detect the specific brain pathologies at a very early stage. Restricted diffusion in basal ganglia, thalami along with typical clinical features suggests CJD rather than any other differential diagnosis of T2 hyperintensity in the basal ganglia.

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15 Mittal S, Farmer P, Kalina P et Al: Correlation of diffusion-weighted magnetic resonance imaging with neuropathology in Creutzfeldt-Jakob disease. Arch Neurol 59: 128-134, 2002. 16 Haik S, Dormont D, Faucheux BA et Al: Prion protein deposits match magnetic resonance imaging signal abnormalities in Creutzfeldt-Jakob disease. Ann Neurol 51: 797-799, 2002. 17 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. Am J Neuroradiol 26: 1551-62, 2005. 18 Finizio FS, Corsini A, De Massis P: Creutzfeldt-Jakob disease: Correlation of clinical neuroradiological aspects and detection of 14.3.3. Protein in cerebrospinal fluid. La malattia di Creutzfeldt-Jacob, correlati neuroradiologici e laboratoristici. Rivista di Neuroradiologia 13: 625, 2000. 19 Bergui M, Bradaˇc GB, Zhong JJ et Al: RM con sequenze pesate in diffusione nella malattia di CreutzfeldtJacob. Rivista di Neuroradiologia 14: 51, 2001. 20 Ottina M, Manara R, Amistà P et Al: Diagnosi in vivo di malattia di Creutzfeldt-Jakob: ruolo delle sequenze T2 e DWI. Rivista di Neuroradiologia 16: 66, 2003. 21 Mori H, Aoki S, Abe O et Al: Non-Homogeneous Increased Intensity of the Cortex on Propeller DW MRI in Creutzfeldt-Jakob Disease. Rivista di Neuroradiologia 17: 13, 2004.

Dr Sukalyan Purkayastha Department of Imaging Sciences & Interventional Radiology Sree Chitra Tirunal Institute for Medical Sciences & Technology Trivandrum - 695011 Kerala - India E-mail: [email protected]

Creutzfeldt-jakob disease. The value of diffusion-weighted MRI.

Creutzfeldt-Jakob disease (CJD) is a transmissible, progressive, fatal spongiform encephalopathy. An early and accurate diagnosis is important to prev...
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