Laboratory and imaging studies for the diagnosis of prion disease

Yusei Shiga, Koichi Miyazawa, Atsushi Takeda, Hiroyuki Arai, Katsumi Doh-Ura, Yasuto Itoyama

Research output: Contribution to journalArticlepeer-review

Abstract

We evaluated the diagnostic sensitivity of periodic synchronous discharge (PSD) in EEG, brain specific proteins in CSF such as neuron specific enolase (NSE), 14-3-3 protein, and tau protein, and imaging studies performed by T2-weighted MRI (T2I) and diffusion-weighted MRI (DWI). 36 patients with a mean age of 68.6 years were enrolled. Their diagnostic levels were as follows: seven were definite, 28 were possible, and one was probable who had a disease-specific point mutation of V180I. The diagnostic sensitivities of PSD, NSE, 14-3-3 protein, tau protein, DWI, and T2I were 50% (N = 36), 70% (N = 30), 80.8% (N = 26), 87.5% (N = 16), 92.3% (N = 26), and 42.3% (N = 26), respectively. DWI could revealed the CJD-related lesions earlier than the appearance of PSD. DWI revealed the lesions even in the patients who did not show PSD. For the diagnosis of CJD, DWI and either 14-3-3 protein or tau protein are useful. Using western blot, we detected the protease-resistant PrP in the urine of 11 of 15 CJD patients. We also detected it in three of 25 disease control patients. Differing from previous reports, the detection of a proterase-resistant PrP was not specific to CJD patients. However, the sensitivity was 73.3% and the specificity was 88.9%.

Original languageEnglish
Pages (from-to)810-812
Number of pages3
JournalClinical Neurology
Volume43
Issue number11
Publication statusPublished - 2003 Nov 1

Keywords

  • 14-3-3 protein
  • Creutzfeldt-Jakob disease
  • Diffusion-weighted MRI
  • Tau protein
  • Urine

ASJC Scopus subject areas

  • Clinical Neurology

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