Article Figures & Data
Figures
- Fig 1.
Basal ganglia involvement. The most common finding on initial imaging during the acute presentation was abnormal T2 signal intensity in the basal ganglia. This ranged from subtle (minimal increased T2 compared with adjacent gray matter) to marked signal-intensity changes with associated mass effect. The latter is illustrated in an axial T2 FSE image in a 6-year-old boy (A). Some cases showed a predilection to specific anatomic areas as seen in an axial T2 FSE image in a boy 2 years 4 months of age (B), in whom there was predominance in the globus pallidus with relative sparing of the other areas of the basal ganglia. C, Corresponding DWI image shows a common correlation in which cases of more focal basal ganglia involvement also had positive DWI findings, though sometimes in a different basal ganglia structure, in this case the putamen.
- Fig 2.
Cortical abnormalities. Examples of cortical abnormalities seen in ret+ CM on axial T2 FSE images. Cortical abnormalities were relatively confluent in some cases as seen in a boy 2 years 11 month of age (A), in whom all the visualized gray matter has increased T2 signal intensity and is diffusely thickened (>5 mm). The cortical abnormalities were distinctly multifocal in others as seen in a girl of 6 years 8 month of age (B), in whom there are patchy areas of involvement and areas of relative sparing. In most cases, the signal-intensity abnormalities and cortical thickening were not confined to typical arterial vascular distributions.
- Fig 3.
Patterns of cortical involvement. The patterns of affected cortical parenchyma generally fell into 1 of 3 distinct types as seen on the EPI DWI images: 1) diffuse; 2) posterior predominance, as is illustrated in a girl of 1 year 3 months of age (A); and 3) frontal predominance, as evident in a girl 3 years 6 months of age (B).
- Fig 4.
Asymmetric hemispheric cortical involvement. Occasionally markedly asymmetric hemispheric cortical involvement was evident. The cortical involvement tended to be confluent and associated with DWI abnormalities such as in an axial T2 FSE (A) and an axial EPI DWI in a girl, 2 years 7 months of age (B). Another example is seen in an axial T2 FSE (C), and an axial EPI DWI (D) in a boy 1 year 11 months of age. Note the associated midline shift. These patients also illustrate how children with CM frequently have constellations of findings seen in ret+ CM but, by no means are all in the same patients at the same time. For example, A and B above have subcortical white matter sparing on the T2 images, while in C and D, no subcortical white matter was involved. Also note how A and B show positive restricted diffusion in the right hemispheric white matter on the DWI images in the absence of significantly high T2 signal-intensity changes.
- Fig 5.
Distribution of white matter changes. There were 2 distinct patterns of white matter involvement: 1) primarily subcortical (A), a coronal T2 FSE in a boy 1 year 6 months of age; and 2) primarily peritrigonal (B), a coronal T2 FSE in a girl 2 years 6 months of age. These frequently coexisted, but subcortical involvement predominated.
- Fig 6.
White matter DWI abnormalities. Subcortical white matter changes had corresponding positive DWI findings, which tended to closely follow theT2 signal-intensity abnormalities. The restricted diffusion was generally confluent and not associated with positive cortical DWI findings. We present 2 representative cases: an axial T2 FSE (A) and an axial EPI DWI (B) in a 7-year-old boy.
- Fig 7.
Posterior fossa involvement. Another common finding in ret+ CM was abnormal T2 signal intensity in the posterior fossa. This ranged from diffuse involvement of the cerebellar cortex with increased parenchymal volume to multifocal areas of cerebellar involvement, including white matter tracts, with localized mass effect as evident in a coronal T2 FSE (A) and an axial T2 FSE (B) in a boy 1 year 2 months of age. The areas and extent of involvement were unrelated to whether there was overall sparing of the posterior fossa structures compared with the presence of supratentorial disease.
Tables
Frequency and characteristics of brain MRI abnormalities seen in children with ret+ versus ret− CM
Characteristics Ret− (n = 32) Ret+ (n = 120) Fisher P Value OR (95% CI) Age (mean in months) 55.1 (27.9) 48.6 (27.6) .23 – Sex (% male) 40.6 54.2 – 1.72 (0.78–3.81) Increased cerebral volume ≥4 7* 57* .01* 3.23 (1.30–8.04)* White matter T2 abnormalities 13* 86* <.005* 3.7 (1.6–8.3)* White matter DWI abnormalities 11 54 .32 1.6 (0.7–3.5) T2 cortical abnormalities 9* 74* .001* 4.1 (1.7–9.7)* Cortical DWI abnormalities 5 25 .62 1.4 (0.5–4.1) Pontine T2 changes 9 57 .07 2.3 (0.9–5.4) T2 changes 15* 89* .005* 3.3 (1.5–7.3)* Basal ganglia involvement 12* 101* <.00001* 8.9 (3.7–21.1)* Basal ganglia DWI abnormalities 4* 48* <.01* 4.7 (1.5–14.2)* Thalamic involvement 10* 77* .001* 3.9 (1.7–9.1)* Corpus callosum T2 abnormalities 6* 59* <.005* 4.2 (1.6–10.9)* Corpus callosum DWI abnormalities 6* 52* <.05* 3.3 (1.3–8.6)* Splenium predominance 5 38 .08 2.5 (0.9–7.0) Posterior fossa DWI abnormalities 0* 6* .34* Undefined Posterior fossa signal abnormalities 7* 59* <.01* 3.5 (1.4–8.6)* Pre-existing abnormality 5 14 .76 0.7 (0.2–2.2) -
Note:—– indicates not applicable;
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↵* statistical significance.
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