Volume 21, Number 5—May 2015
CME ACTIVITY - Perspective
Recent US Case of Variant Creutzfeldt-Jakob Disease—Global Implications
||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 updated: April 19, 2015
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