Figure 1.

Brain MRI performed at 7 months of age (a–h). (a–d) Axial T2-weighted images show diffuse high signal of almost the entire brainstem, including the hilum of the inferior olivary nuclei (white thin arrows) and the central tegmental tracts (black thin arrows) with only sparing of the most dorsal tegmental portion. The cerebellar white matter is involved (asterisks) as well as the middle cerebellar peduncles (black arrowheads) and supratentorial white matter. Partial myelination of the pyramidal tracts at the PLIC is visible (empty black arrows). (e) Axial T1-weighted image reveals low signal of the lobar white matter with a posteroanterior gradient. (f, g) Axial diffusion weighted images (DWI) images show restricted diffusion within both corticospinal tracts (black thick arrows). (h) Sagittal T2-weighted image reveal diffuse signal changes of the cervical spinal cord (white arrowheads).