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Fig 1. A, T2-weighted sagittal section in patient 9 shows thinning of the medulla oblongata and spinal cord with hyperintensity in the medulla; arrow indicates its posterior concave profile. B–D, Axial T2-weighted sections (thickness 3 mm) in the same patient demonstrate increased signal intensity involving the hilum of the dentate nuclei (arrow on the left side), the medial lemniscus (arrowhead, B), and the corticospinal tracts (arrowheads on the left side) in the medulla (C and D). In patient 11 (E), axial spinal cord T2-weighted section at the C1-C2 level shows severe atrophy with mild changes in signal intensity.