Probable variant Creutzfeldt-Jakob disease in Asia: A case report from Taiwan and review of two prior cases

Chih Wen Yang, Jong Ling Fuh, Shuu Jiun Wang*, Jiing Feng Lirng, Chih Chao Yang, Shih Jung Cheng

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

New variant Creutzfeldt-Jakob disease (vCJD) was first identified in the UK in 1996, and was causally linked to bovine spongiform encephalopathy. Herein we report the first case of vCJD in Taiwan: a 34-year-old man who had lived in the UK between 1989 and 1997. The patient presented with depression, irritability, personality change, painful feet and allodynia, followed by gait ataxia and cognitive impairment. Electroencephalograms did not show the typical appearance of sporadic CJD. The cerebrospinal fluid 14-3-3 protein immunoassay was negative. Brain magnetic resonance imaging revealed high signal lesions involving bilateral caudate nuclei, left lentiform nucleus, bilateral dorsomedial thalami and pulvinar on fluid-attenuation inversion recovery, T2- and diffusion-weighted imaging. Prion protein gene analysis showed homozygous for methionine at codon 129. The patient developed akinetic mutism at 16 months and died at 28 months after onset. The clinical presentation and neuroimaging findings were compatible with the vCJD cases reported since 1996, and met the World Health Organization Case Definition for probable vCJD. In this communication, we also review two other cases of vCJD in Asia. All three cases were assumed as imported cases from the UK because of the residential or travel history of the patients.

Original languageEnglish
Pages (from-to)652-658
Number of pages7
JournalPsychiatry and Clinical Neurosciences
Volume64
Issue number6
DOIs
StatePublished - Dec 2010

Keywords

  • magnetic resonance imaging
  • prion
  • pulvinar sign
  • variant Creutzfeldt-Jakob disease

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