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Volume 21, Number 5—May 2015
CME ACTIVITY - Perspective

Recent US Case of Variant Creutzfeldt-Jakob Disease—Global Implications

Atul MaheshwariComments to Author , Michael Fischer, Pierluigi Gambetti, Alicia Parker, Aarthi Ram, Claudio Soto, Luis Concha-Marambio, Yvonne Cohen, Ermias D. Belay, Ryan A. Maddox, Simon Mead, Clay Goodman, Joseph S. Kass, Lawrence B. Schonberger, and Haitham M. Hussein
Author affiliations: Baylor College of Medicine, Houston, Texas, USA (A. Maheshwari, A. Parker, A. Ram, C. Goodman, J.S. Kass); Harris Health System, Houston (A. Maheshwari, A. Parker, A. Ram, J.S. Kass); Texas Department of State Health Services, Austin, Texas, USA (M. Fischer); Case Western Reserve University School of Medicine, Cleveland, Ohio, USA (P. Gambetti, Y. Cohen); University of Texas Medical School at Houston, Houston (C. Soto, L. Concha-Marambio); Universidad de los Andes, Santiago, Chile (L. Concha-Marambio); Centers for Disease Control and Prevention, Atlanta, Georgia, USA (E.D. Belay, R.A. Maddox, L.B. Schonberger); University College London Institute of Neurology, London, UK (S. Mead); HealthPartners Clinics & Services, St. Paul, Minnesota, USA (H.M. Hussein)

Main Article

Table 2

World Health Organization diagnostic criteria for vCJD and sCJD*

Diagnosis vCJD sCJD
Progressive psychiatric disorder lasting >6 mo with no alternate explanation; at least 4 of the following: early psychiatric symptoms, persistent pain and/or dysesthesia, ataxia, chorea/ dystonia/myoclonus, and dementia; EEG without periodic sharp wave complexes typical for sCJD
Progressive dementia <2 y duration (typically <6 mo); at least 2 of the following: myoclonus, visual or cerebellar disturbance, pyramidal or extrapyramidal dysfunction, akinetic mutism; EEG atypical (not showing periodic sharp wave complexes) or not done
Probable Meets criteria for possible vCJD plus: Meets criteria for possible sCJD plus:

EEG not consistent with sCJD; and bilateral pulvinar high signal on MRI of brain OR progressive psychiatric disorder lasting >6 mo with no alternate explanation; and positive tonsil biopsy
Typical EEG findings (generalized periodic sharp wave complexes at ≈1 Hz); and/or positive 14-3-3 assay in CSF and clinical duration leading to death in <2 y
Definite Neuropathologic confirmation of vCJD Neuropathologic confirmation of sCJD

*See (11,13). CJD, Creutzfeldt-Jakob disease; CSF, cerebrospinal fluid; EEG, electroencephalography; MRI, magnetic resonance imaging; sCJD, sporadic CJD; vCJD, variant CJD.

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Page created: April 19, 2015
Page updated: April 19, 2015
Page reviewed: April 19, 2015
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