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Clinical Images  |   January 2019
Creutzfeldt-Jakob Disease
Author Notes
  • Financial Disclosures: None reported. 
  • Support: None reported. 
  •  *Address correspondence to Viet H. Le, DO, MS Department of Neurology, Garden City Hospital, 6245 Inkster Road, Garden City, MI 48135-4001. Email: viethle.do@gmail.com
     
Article Information
Geriatric Medicine / Imaging / Neuromusculoskeletal Disorders / Clinical Images
Clinical Images   |   January 2019
Creutzfeldt-Jakob Disease
The Journal of the American Osteopathic Association, January 2019, Vol. 119, 63. doi:https://doi.org/10.7556/jaoa.2019.010
The Journal of the American Osteopathic Association, January 2019, Vol. 119, 63. doi:https://doi.org/10.7556/jaoa.2019.010
A 78-year-old man presented to the emergency department with sudden-onset aphasia and right upper extremity weakness. Neurologic examination revealed decreased right upper extremity motor strength, hyperreflexia, and akinetic mutism. A magnetic resonance image revealed hyperintensity of the cortical gyri in the fronto-occipital lobes, called cortical ribboning (image). Tau proteins (30,074 pg/mL) were found in the cerebrospinal fluid. Real-time quaking-induced conversion assay detected prion proteins. A 14-3-3 protein level was inconclusive due to blood contaminant. A diagnosis of Creutzfeldt-Jakob disease (CJD) was made. 
Diagnostic criteria for sporadic CJD require electroencephalography findings of periodic sharp wave complexes, the presence of 14-3-3 protein, or cortical ribboning on magnetic resonance imaging plus clinical features, rapidly progressive dementia, and at least 2 of the following symptoms: myoclonus, visual or cerebellar dysfunction, pyramidal/extrapyramidal features, and akinetic mutism.1 The patient exhibited cortical ribboning, dementia, akinetic mutism, and hyperreflexia, which led to the diagnosis. Magnetic resonance imaging is a useful, noninvasive modality that has 91% sensitivity, 95% specificity, and 94% accuracy in differentiating CJD from other dementias.2 
References
Manix M, Kalakoti P, Henry M, et al Creutzfeldt-Jakob disease: updated diagnostic criteria, treatment algorithm, and the utility of brain biopsy. Neurosurg Focus. 2015;39(5):E2. doi: 10.3171/2015.8.FOCUS15328 [CrossRef] [PubMed]
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(6):1551-1562. [PubMed]