A Unique Case of Sporadic Creutzfeldt-Jacob Disease Presenting as Progressive Supranuclear Palsy

Munehisa SHIMAMURA, Eiichiro UYAMA, Teruyuki HIRANO, Tatsuhumi MURAKAMI, Shuji MlTA, Tetsuyuki KlTAMOTO, Makoto UCHINO
2003 Internal medicine (Tokyo. 1992)  
Wereport 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 myoclonusnor periodic synchronous discharge was observed. Diffusionweighted 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
more » ... n of prion protein of a patchy/perivacuolar type with spongiform degeneration. Thus, the phenotype of sporadic CJD seems variable and DWIshould be performed, even in atypical cases lacking the characteristics of CJD. (Internal Medicine 42: 195-198, 2003)
doi:10.2169/internalmedicine.42.195 fatcat:xjigro67mvh3xdsypylu6gtpsq