case_images: PMC10989518_01
case_images: Brain MRI at presentation shows cortical DWI hyperintensities "cortical ribboning" (A,B) with ADC hypointensity (C,D) over the corresponding cortex. Brain MRI at follow-up shows spreading of the cortical restriction in DWI (E,F) and ADC (G,H) currently involving the left frontal, parietal, temporal, and occipital lobes.
case_images: fneur-15-1367361-g001.jpg
case_images: PMC10989518_01_fneur-15-1367361-g001.jpg
case_images: fig1
case_images: An initial diagnosis of ischemic stroke was done following a magnetic resonance imaging (MRI), showing a cortical hyperintensity in the diffusion-weighted imaging (DWI) with a mild decrease in apparent diffusion coefficient (ADC) in the left occipital lobe (Figures 1A-D). A review of the initial MRI was consistent with "cortical ribboning" regarded as a typical imaging feature of sCJD (Figures 1A-D). In a second MRI carried out 2 weeks later, the cortical hyperintensities in DWI became more extensive, involving the left frontal, parietal, temporal, and occipital lobes with mild cortical restriction in the right hemisphere (Figures 1E,F), along with decreased ADC over the corresponding cortex (Figures 1G,H).
case_images: EEG shows classic periodic triphasic waves with a slow background.
case_images: fneur-15-1367361-g002.jpg
case_images: PMC10989518_01_fneur-15-1367361-g002.jpg
case_images: fig2
case_images: The EEG showed periodic sharp wave complexes with triphasic waves (TWs), mostly in the occipital lobes with diffuse background slowing (Figure 2).
case_images: Antibody detection in serum and CSF. The result of TBA showed an uncertain neuronal surface antibody. (A) The immunofluorescence signal was detected in positive control (titer 1:1); (B) the immunofluorescence signal could not be detected in serum from the patient; (C) the immunofluorescence signal was detected in positive control (titer 1:50); (D) the immunofluorescence signal was detected in CSF from the patient; and (E,F) the results of CBA showed antibodies against IgLON5 were detected in both the serum (titer 1:300) (E) and CSF (titer 1:100) (F).
case_images: fneur-15-1367361-g003.jpg
case_images: PMC10989518_01_fneur-15-1367361-g003.jpg
case_images: fig3
case_images: Compared with a positive control (titer 1:1) (Figure 3A), no immunofluorescence was detected in serum (Figure 3B); however, compared with a positive control in the CSF (titer 1:50) (Figure 3C), an immunofluorescence signal was also detected in the CSF (titer 1:50) (Figure 3D). Then, cell-based assays (CBAs) were performed showing IgLON5 antibodies in the serum (titer 1:300) (Figure 3E) and the CSF (titer 1:100) (Figure 3F), while other autoantibodies remained negative.