Survival to akinetic mutism state in Japanese cases of MM1-type sporadic Creutzfeldt-Jakob disease is similar to Caucasians

Eur J Neurol. 2011 Jul;18(7):999-1002. doi: 10.1111/j.1468-1331.2010.03185.x. Epub 2010 Aug 16.


Background: It is not known whether the clinical course of Japanese sporadic Creutzfeldt-Jakob disease (sCJD) cases differs from that of Caucasian sCJD cases.

Patients and methods: To investigate the clinical course of Japanese sCJD, clinical findings from 29 patients with Japanese MM1-type sCJD were retrospectively evaluated and compared to Caucasian sCJD findings.

Results: Survival of Japanese MM1-type sCJD up to the time of akinetic mutism state is similar to that of Caucasian subjects. However, the total disease duration of Japanese patients was approximately three times longer.

Conclusions: The present observations indicate that Japanese sCJD cases generally show a longer disease duration because of the longer survival period after reaching the akinetic mutism state.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Age of Onset
  • Aged
  • Aged, 80 and over
  • Akinetic Mutism / ethnology*
  • Akinetic Mutism / etiology*
  • Asian Continental Ancestry Group
  • Creutzfeldt-Jakob Syndrome / complications*
  • Creutzfeldt-Jakob Syndrome / ethnology*
  • Creutzfeldt-Jakob Syndrome / mortality
  • Disease Progression
  • European Continental Ancestry Group
  • Female
  • Humans
  • Male
  • Middle Aged
  • Retrospective Studies
  • Time