E-Pearl of the Week: Diagnosing Creutzfeldt-Jakob Disease

June 20, 2012

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June 19, 2012

Diagnosing Creutzfeldt–Jakob Disease

Creutzfeldt–Jakob disease presents as a rapidly progressive dementia. Noninvasive diagnostic criteria have been accepted by the World Health Organization:

1. Progressive dementia; and
2. Two of the following: myoclonus, pyramidal/extrapyramidal features, visual/cerebellar features, or akinetic mutism
3. One of the following: typical periodic pattern on EEG at any time point during the illness or, if less than 2 years in duration, positive CSF 14–3–3 biomarker
4. Exclusion of other potential conditions

Reference

1. World Health Organization (WHO). Global surveillance, diagnosis, and therapy of human transmissible spongiform encephalopathies: report of a WHO consultation. February 9–11, 1998; Geneva. Available at:
www.who.int/csr/resources/publications/bse/WHO_EMC_ZDI_98_9/en/. Accessed October 6, 2011.

Submitted by: Christopher Newey, D.O., M.S.

Disclosure: Dr. Newey reports no disclosures.

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