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).