PMC5187649_01
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
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Fig1
Strikingly, brain MR imaging revealed a general atrophy of brain parenchyma with remarkable emphasis on the brain stem region (Fig. 1a and b). Here, a manifest gliotic neurodegeneration in the medulla oblongata with predominant loss of pyramidal tracts presented as "tadpole" appearance - a radiological sign exclusively described in association with Alexander's disease (Fig. 1a). Even more prominently, pattern of brain stem degeneration resembled "kissing swans" in coronal sections (Fig. 1c). Importantly, no contrast enhancement could be observed in the brain stem or any other part of the brain following Gadolinium administration (Fig. 1d), indicating blood-brain barrier integrity. Furthermore, a marked atrophy of the upper cervical spinal cord was detected (Fig. 1e). 