A unique case of sporadic Creutzfeldt-Jacob disease presenting as progressive supranuclear palsy

Munehisa Shimamura, Eiichiro Uyama, Teruyuki Hirano, Tatsuhumi Murakami, Shuji Mita, Tetsuyuki Kitamoto, Makoto Uchino

Research output: Contribution to journalArticlepeer-review

21 Citations (Scopus)

Abstract

We report a Japanese case of sporadic Creutzfeldt-Jakob disease (CJD) presenting as progressive supranuclear palsy. For 2 years after onset, neurological deficits had slowly progressed but neither myoclonus nor periodic synchronous discharge was observed. Diffusion-weighted image (DWI) showed unique high signal lesions in the bilateral frontal cortex, left parietooccipital and occipital cortices, but there was nearly no change eight months later. Needle biopsy revealed deposition of prion protein of a patchy/perivacuolar type with spongiform degeneration. Thus, the phenotype of sporadic CJD seems variable and DWI should be performed, even in atypical cases lacking the characteristics of CJD.

Original languageEnglish
Pages (from-to)195-198
Number of pages4
JournalInternal Medicine
Volume42
Issue number2
DOIs
Publication statusPublished - 2003 Feb 1

Keywords

  • Creutzfeldt-Jakob disease
  • Diffusion-weighted image
  • Genetics
  • Pathology
  • Progressive supranuclear palsy
  • Single photon emission computed tomography

ASJC Scopus subject areas

  • Internal Medicine

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