Creutzfeldt-Jakob Disease
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Viet H. Le
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
1. Manix M , KalakotiP, HenryM, 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.FOCUS15328Search in Google Scholar PubMed
2. Young GS , GeschwindMD, FischbeinNJ, 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.Search in Google Scholar
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Articles in the same Issue
- EDITORIAL
- Driving Progress and Innovation Through Research
- STILL RELEVANT?
- Jeanette “Nettie” Bolles: The First Female DO
- ORIGINAL CONTRIBUTION
- Correlation Between Physiologic and Osteopathic Measures of Sympathetic Activity in Women With Polycystic Ovary Syndrome
- BRIEF REPORT
- Tolerance of Rib Raising Among Hospitalized Patients: A Pilot Study
- REVIEW
- Nonpharmacologic Management of Symptoms in Females With Polycystic Ovary Syndrome: A Narrative Review
- Structural and Functional Aberrations of the Auditory Brainstem in Autism Spectrum Disorder
- JAOA/AACOM MEDICAL EDUCATION
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- Dry Needling–Induced Pneumothorax
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