[Laboratory and imaging studies for the diagnosis of prion disease].
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 protease-resistant PrP was not specific to CJD patients. However, the sensitivity was 73.3% and the specificity was 88.9%.
Shiga
Yusei
Y
Department of Neurology, Tohoku University School of Medicine.
Miyazawa
Koichi
K
Takeda
Atsushi
A
Arai
Hiroyuki
H
Doh-ura
Katsumi
K
Itoyama
Yasuto
Y
D048948:14-3-3 Proteins; D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers / Q000134:cerebrospinal fluid; D007562:Creutzfeldt-Jakob Syndrome / Q000145:classification / Q000175:diagnosis*; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D010751:Phosphopyruvate Hydratase / Q000134:cerebrospinal fluid; D012680:Sensitivity and Specificity; D014446:Tyrosine 3-Monooxygenase / Q000134:cerebrospinal fluid*; D016875:tau Proteins / Q000134:cerebrospinal fluid
D048948:14-3-3 Proteins; D015415:Biomarkers; D016875:tau Proteins; D014446:Tyrosine 3-Monooxygenase; D010751:Phosphopyruvate Hydratase