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Annals of Indian Academy of Neurology
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IMAGES IN NEUROLOGY
Year : 2009  |  Volume : 12  |  Issue : 1  |  Page : 52-53
 

Creutzfeldt-Jakob disease: Importance of early magnetic resonance imaging


Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi - 110 029, India

Date of Submission21-Apr-2008
Date of Decision19-Jun-2008
Date of Acceptance29-Jun-2008

Correspondence Address:
K S Madhusudhan
Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.48859

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How to cite this article:
Madhusudhan K S, Das CJ. Creutzfeldt-Jakob disease: Importance of early magnetic resonance imaging. Ann Indian Acad Neurol 2009;12:52-3

How to cite this URL:
Madhusudhan K S, Das CJ. Creutzfeldt-Jakob disease: Importance of early magnetic resonance imaging. Ann Indian Acad Neurol [serial online] 2009 [cited 2020 Nov 23];12:52-3. Available from: https://www.annalsofian.org/text.asp?2009/12/1/52/48859


A 72-year-old male presented with ten days history of altered sensorium, involuntary movement of limbs, and progressive dementia. There was no history of fever or vomiting. On examination, the patient was disoriented, the muscle tone was increased, and tendon reflexes were exaggerated. The Babinski's sign was positive bilaterally. There were no signs of meningeal irritation or cerebellar signs. The EEG was normal. MRI done as a part of routine checkup showed diffuse hyperintensity in the cortex of bilateral frontal and parietal lobes and in bilateral caudate nuclei, globus pallidi, and putamina on diffusion weighted [DWI; [Figure 1A] and [Figure 1B]] and fluid-attenuated inversion recovery (FLAIR) images [Figure 1C] with restriction of diffusion. Imaging appearance was typical for CJD in the clinical setting. Subsequent CSF analysis for 14-3-3 protein was positive, confirming the diagnosis.

CJD is a transmissible, rapidly progressive, invariably fatal neurodegenerative disorder. [1] It is caused by the accumulation of abnormal prion protein in the neurons resulting in their spongiform degeneration. [1] Clinical diagnosis is often difficult and needs exclusion of other diseases producing similar symptoms. MRI, especially the DWI sequence, plays a vital role in suggesting an early diagnosis. It characteristically shows symmetrical T2 hyperintensities with restricted diffusion in bilateral cerebral cortices and basal ganglia. [2],[3] Confirmation of the disease requires demonstration of periodic sharp wave complexes on EEG or 14-3-3 protein in CSF. [4] The disease is transmissible and early diagnosis is helpful in appropriate patient management. The disease is fatal and no treatment is currently available.

 
   References Top

1.Masters CL, Harris JO, Gajdusek DC, Gibbs CJ Jr, Bernoulli C, Asher DM. Creutzfeldt-Jakob disease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann Neurol 1979;5:177-88.  Back to cited text no. 1  [PUBMED]  
2.Young GS, Geschwind MD, Fischbein NJ, Martindale JL, Henry RG, Liu S, 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.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Shiga Y, Miyazawa K, Sato S, Fukushima R, Shibuya S, Sato Y, et al. Diffusion-weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt-Jakob disease. Neurology 2004;63:443-9.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.World Health Organization. Human transmissible spongiform encephalopathies. Wkly Epidemiol Rec 1998;73:361-5.  Back to cited text no. 4    


    Figures

  [Figure 1A], [Figure 1B], [Figure 1C]



 

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