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

June 20, 2012


Interested in submitting an e–Pearl? Click here!

Brought to you by the Resident and Fellow Section of Neurology®.

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


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.

For more clinical pearls and other articles of interest to neurology trainees, visit www.neurology.org and click on the link to the Resident and Fellow Pages. Click here to visit the E–Pearl of the Week Archive.

Click here to listen to this week's Neurology® Podcast.