Article Figures & Data
Figures
- Fig 1.
Leukoencephalopathy. A, Case 19. Axial spin-echo T2-weighted (1.5T, TR = 2554 ms, TE = 100 ms) MR image shows a faint T2 hyperintensity involving the cerebral white matter in a diffuse pattern. Calcifications are seen in the lobar white matter as focal hypointensities (arrows). B and C, Axial T2- (1.5T, turbo spin-echo, TR = 5022 ms, TE = 100 ms) (B) and T1-weighted images (1.5T, inversion recovery, TR = 3430 ms, TE = 15 ms, TI = 400 ms) (C), with the same parameters in case 10, demonstrate extensive cerebral white matter abnormalities, showing an anteroposterior gradient. The T1-weighted images better highlight areas of cavities in the frontal lobes, whereas the signal-intensity properties of the involved white matter are almost identical to those of the liquoral spaces (arrows).
- Fig 2.
Cerebral calcifications. A, Axial nonenhanced CT image of case 1 shows numerous punctuate calcifications within the basal ganglia and the cerebral white matter, a pattern typical in patients with AGS. B, Contrast-enhanced CT scan (case 19) shows large calcifications in the white matter. Although the CT examination was performed in the acute phase of the disease, no signs of contrast enhancement are seen. Atrophy, microcephaly, and areas of hypoattenuation in the periventricular white matter are also evident.
- Fig 3.
Coronal fast spin-echo T2-weighted MR image (1.5T, TR = 5022 ms, TE = 100 ms) of case 10 shows a diffuse signal-intensity abnormality of the cerebral lobar white matter. Note that the cerebellar white matter and the optic radiations (arrows) are spared, whereas the subcortical U-fibers are involved. The cortex shows an even thickness. Severe cerebral atrophy is evident.
- Fig 4.
Correlation between age at onset and the severity of leukoencephalopathy involving the frontal and temporal lobes (leukoencephalopathy score: 1 = mild; 2 = moderate; 3 = severe; 4 = presence of cystic degeneration). Mths indicate months.
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