case_images: PMC6629179_01
case_images: MR images and SPECT obtained 15 months after the onset of symptom.. T1-weghted MR images (a-c); diffusion-weighted images (d-f), an easy Z-score (eZIS) analysis of 9mTc-etyhlcysteinate dimer-single photon emission computed tomography (99mTc-ECD-SPECT) image (g). The Z-score scale of 2.0 to 6 is indicated by a green to red (lower regional cerebral blood flow (rCBF)) color gradient in panel g. R: right side; L: left side. T1-weighted MRI showed bilateral frontotemporal atrophy and symmetric enlarged lateral ventricles predominantly posterior horn due to occipital atrophy (a-c). Diffusion-weighted MRI showed cortical hyper-intensities in the bilateral frontal, temporal parietal and occipital cortices without obvious signal changes in the basal ganglia 15 months after the initial symptom (d-f). An eZIS analysis of 99mTc-ECD-SPECT showed decreased rCBF in the bilateral frontal, parietal, and right posterior cortices 15 months after the onset (g).
case_images: kprn-13-01-1631680-g001.jpg
case_images: PMC6629179_01_kprn-13-01-1631680-g001.jpg
case_images: F0001
case_images: T1-weighted MRI showed bilateral frontotemporal atrophy and symmetrically enlarged lateral ventricles of the posterior horn due to occipital atrophy (Figure 1a-c). 1.5-Tesla diffusion-weighted MRI (DW-MRI) showed cortical hyper-intensities in the bilateral frontal, temporal parietal and occipital lobes without obvious signal changes in the basal ganglia 15 months after the initial symptom (Figure 1d-f). An easy Z-score (eZIS) analysis (Fujifilm RI Pharma, Tokyo, Japan) of 99mTc-etyhlcysteinate dimer-single photon emission computed tomography (99mTc-ECD-SPECT) showed decreased regional cerebral blood flow (rCBF) in the bilateral frontal, parietal, and right posterior cortices, 15 months after the onset (Figure 1g).
case_images: Western blot analysis of protease-resistant PrP.. Western blot analysis using 3F4 antibodies (a), Tohoku-1 (b), or Tohoku-2 (c). Brain homogenate sample from MM1 patient (a), the current patient (b), or MM1 + 2 patient (c). Panels showed type 2 PrPsc, with 19 kDa unglycosylated band in the current patient.
case_images: kprn-13-01-1631680-g003.jpg
case_images: PMC6629179_01_kprn-13-01-1631680-g003.jpg
case_images: F0003
case_images: Western blot analysis using 3F4 antibody revealed the presence of type 2 PrPsc, as 19 kDa unglycosylated band (Figure 3a). Moreover, Western blot analyzes using type 2 PrPsc-specific antibody showed the presence of apparent positive bands, whereas, no positive bands were observed with type 1 PrPsc-specific antibody (Figure 3b, 3c).