Fig. 1

Brain stem degeneration presents in brain MRI. Sagittal (a) and transversal (b) T2-weighted brain MRI indicates gliotic neurodegeneration in the medulla oblongata with predominant loss of pyramidal tracts. Magnification of inlay (c) uncovers pathologic brain stem formation reminiscent of ‘kissing swans’. d Transversal T1-weighted sectioning of the brain stem (arrow) after Gadolinium administration. No contrast enhancement is detectable. e Sagittal T2-weighted cervical spine MRI shows atrophy of the upper cervical spinal cord in addition to medulla oblongata atrophy. f Transversal T2-weighted brain MRI indicates putative periventricular rim-sign and global brain atrophy. g Brain MRI-angiography reveals normal intracranial vascular status without indication of stenosis