Article Figures & Data
Figures
- 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).
- 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 ×40). F, In the microphotograph, Bunina bodies are observed in the spinal motor neurons (H&E, original magnification ×600). 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 ×400).
Tables
Clinical and MR imaging findings in patients with ALS
Patient No. Age (y) Sex Duration Before MRI (y) Dementia (HDS-R) Bulbar Symptoms Upper Motor Neuron Symptoms Lower Motor Neuron Symptoms, Upper Extremities Lower Motor Neuron Symptoms, Lower Extremities Atrophy Frontotemporal Lobes* HI Anterior Temporal White Matter HI Bilateral Precentral White Matter HI Bilateral Corticospinal Tracts HI Bilateral Precentral Cortices Small Infarction or Unidentified Bright Objects 1 58 M 1 +, 17/30 + − + + 2, T, bilateral +, bilateral − − + − 2 65 M 6 +, 14/30 + − + + 3, T, bilateral +, bilateral + − − + 3 71 F 2 +, 9/30 + − + + 2, Fr, bilateral +, bilateral − − − + Subtotal 64.7 ± 6.5 3.0 ± 2.6 100% 2.33 100% 33.3% 0% 33.3% 66.7% 4 46 M 1 − + + + + 1 − − − − − 5 50 F 6 − + + + + 1 − − − + − 6 54 F 6 − − + + + 1 − − − − − 7 55 F 2 − + + + + 2, Fr, bilateral − + + + − 8 57 M 1 − + + + + 1 − − − − + 9 57 M 3 − + − − − 1 − − − − + 10 58 F 1 − + + + + 1 − + + + + 11 60 F 1 − + + + − 1 − − + + − 12 62 F 0 − + + + − 1 − + + + + 13 62 F 2 − + + + + 2, Fr, bilateral − − + + + 14 62 M 4 − + + + + 2, T, bilateral − − − + + 15 62 M 6 − + + + − 1 − − − − + 16 65 M 7 − + + + − 2, T, bilateral − − − − − 17 67 M 2 − − + − + 1 − + − + − 18 69 M 8 − + + + + 1 − − − + + 19 70 F 2 − + + + + 1 − + − + + 20 71 M 5 − + + + + 2, T, bilateral − + − + + 21 74 F 1 − + + + + 1 − + − − + 22 74 M 2 − − − + + 2, Fr, bilateral − − − − + 23 74 M 4 − + + + + 1 − + + + + 24 74 M 4 − + − + + 1 − + − + + Subtotal 63.0 ± 8.3 3.2 ± 2.3 0% 1.33 0% 42.9% 28.6% 61.9% 66.7% Total 63.2 ± 8.0 3.2 ± 2.3 12.5% 1.46 12.5% 41.7% 37.5% 58.3% 66.7% Note:—HI indicates hyperintensity; Fr, frontal dominance; T, temporal dominance; +, present; −, absent.
* 3 indicates severe; 2, moderate; 1, mild or absent.
