Figure 1

Figure 1

Brain and spinal cord imaging. At initial presentation: (A) MRI cervical spine sagittal T2-weighted image with hyperintense signal involving the central gray matter, dorsal and lateral tracts of the cervical and thoracic spinal cord (yellow arrow). (B) MRI thoracic spine axial T2-weighted image with hyperintense signal involving primarily gray matter (yellow arrow). (C) MRI cervical spine axial post-gadolinium T1-weighted image with contrast enhancement of the dorsal white matter tracts (yellow arrow). Follow-up at 6 months: (D) MRI cervical spine sagittal T2-weighted image with re-demonstration of central gray matter hyperintensity of the cervical and thoracic spinal cord with associated volume loss (white arrow). (E) MRI cervical spine axial T2-weighted image with increased hyperintense signal involving primarily gray matter and volume loss within the gray matter tracts (white arrow). (F) MRI brain axial T2-weighted FLAIR image with diffuse parenchymal volume loss and nearly symmetric bilateral periventricular and deep white matter signal abnormality in the frontal, parietal, and occipital lobes (white arrows). Obtained 2 weeks before death: (G) MRI cervical spine sagittal T2-weighted image with recurrent non-enhancing hyperintense signal involving primarily the dorsal central aspect of the spinal (red arrow). (H) MRI brain axial T1-weighted imaging post-gadolinium with contrast enhancement involving the optic chiasm, prechiasmatic optic nerves, and bilateral optic tracts within the suprasellar region (red arrows). (I) MRI brain axial T2-weighted FLAIR image with progressive bilateral periventricular white matter signal abnormality with central areas of white matter necrosis (red arrows).