AJDRAJNR - American Journal of Neuroradiology

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Symmetric Temporal Abnormalities on MR Imaging in Amyotrophic Lateral Sclerosis with Dementia

H. Moria,b, A. Yagishitab, T. Takedac and T. Mizutanid

a From the Department of Radiology, Graduate School of Medicine, University of Tokyo, Tokyo, Japan
b Department of Neuroradiology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan
c Department of Neuropathology, Tokyo Metropolitan Institute for Neuroscience, Tokyo, Japan
d Department of Neuropathology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan


Figure 1
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Fig 1. Patient 1, ALSD. A, Transverse SE T2WI shows symmetric temporal atrophy and symmetric hyperintensity (arrows) in the subcortical white matter on the medial side of the anterior temporal lobes. B, Transverse SE T2WI obtained at the level of high convex shows hypointensity along the precentral cortices (arrowheads).


Figure 2
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Fig 2. Patient 2, ALSD. A, Coronal FSE T2WI obtained at the level of the temporal tip shows symmetric temporal atrophy and symmetric hyperintensity (arrowheads) in the anteromedial temporal white matter. B, Coronal FSE T2WI obtained at the level of the temporal tip also shows symmetric hyperintensity (arrowheads) in the subcortical white matter of the frontal base and insula. C, Transverse SE T2WI shows symmetric hyperintensity (arrows) in the subcortical white matter of the anterior temporal lobes. D, Photograph of postmortem coronal specimen (myelin-stained section) obtained at the level of the temporal tip shows loss of myelinated fibers in the right anteromedial temporal lobe (arrowheads). Arrow indicates the top of the image. E, Microphotograph of the cortex in the temporal pole shows that the superficial layers II and III exhibit spongiosis (arrows) (H&E, original magnification x40). F, In the microphotograph, Bunina bodies are observed in the spinal motor neurons (H&E, original magnification x600). G, In the microphotograph of the dentate gyrus, ubiquitin-positive cytoplasmic inclusions are present in the granular cells (arrowheads) (immunostaining with anti-ubiquitin, original magnification x400).