Article Figures & Data
Figures
- Fig 1.
Patient 6 at 6 years of age (CS I). A, Axial CT scan at the basal ganglia level shows severe bilateral calcifications in the putamen. Subtle calcifications are seen in the frontal and parietal cortex at the depths of the sulci (arrows). B, Axial CT scan of the posterior fossa shows bilateral slight calcifications in the dentate nuclei (arrows).
- Fig 2.
Patient 14 (CS II) at 2 years of age. A, Axial CT scan at the basal ganglia level shows bilateral calcifications in the cortex at the depths of the sulci in the parietal regions (arrows), in addition to mild bilateral putaminal calcifications (arrowheads). B, Coronal reformatted CT scan shows bilateral calcifications in the cortex at the depths of the sulci in the frontal regions (arrows). C, Axial maximum-intensity-projection reformation of the CT scan (A) shows calcified leptomeningeal vessels, notably in the right insular region (arrow).
- Fig 3.
Patient 10 (CS I) at 4 (A and B) and 13 (C and D) years of age. A and C, Midline sagittal T1-weighted images demonstrate the progressive development of generalized cerebral and cerebellar atrophy, which is very severe at 13 years of age, with substantial corpus callosum and brain stem thinning. Note the thickened cranial vault and frontal and sphenoid sinus dilation at 13 years of age related to brain atrophy. B and D, Axial T2-weighted images show only a slight ventricular dilation at 4 years of age but considerable atrophy of the cortex as well as the white matter at 13 years of age, with increased ventricular dilation.
- Fig 4.
Patient 18 (COFS) at 3 months (A) and 2 years (B) of age. A, Coronal T2-weighted image shows cerebellar hypoplasia. B, Coronal reformatted T1-weighted image demonstrates on follow-up that the cerebellum is now extremely atrophic.
- Fig 5.
Axial T2-weighted images of patient 18 (COFS) at 3 months (A) and 2 years (B–D) of age. A, High signal intensity is present in the posterior limb of the internal capsule, consistent with an early lack of myelin. B–D, At 2 years of age, the myelination in the posterior fossa is normal (B), but no myelin is seen in the supratentorial white matter (C and D), which shows diffuse mild hyperintensity, including in the internal and external capsules (C). This aspect is consistent with hypomyelination. Note also the progressive cerebral atrophy.
- Fig 6.
Axial T2-weighted images of patient 1 (CS III) at 15 years of age. A, The cerebellum and the brain stem are fully myelinated. B, Myelination is also seen in the corpus callosum and the posterior limb of the internal capsule. The T2 hyperintensity lining of the medial part of the right internal capsule is probably the result of its progressive atrophy. C, The semioval center shows a punctuate appearance (arrow), with hypointense foci within the hyperintense white matter, reflecting preserved perivascular myelin. This corresponds to the tigroid leukodystrophy found in CS neuropathology.
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