MR Imaging of Tuberous Sclerosis in Neonates and Young Infants
Yvonne Barona and
A. James Barkovich
,a
a From the Department of Radiology, Section of Neuroradiology, University of California at San Francisco (Y.B., A.J.B.); and Radiologische Abteilung, Staedtisches Krankenhaus Kiel, Germany (Y.B.).
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FIG 1. Neonate, 4 days. Sagittal SE T1-weighted MR image (600/8/2) shows white matter anomalies as punctate hyperintensities (two marked by long arrows) radiating toward ventricles, several subependymal nodules (one marked by curved arrow) in left atrium, and incidental postnatal findings of hyperintense subdural hemorrhage overlying cerebellum and parietal caput succedaneum (not marked).
FIG 2. Neonate, 7 days.
A, Sagittal SE T1-weighted MR image (600/15/2) shows left-hemispheric large transmantle dysplasia (short arrows) as hyperintense area extending from frontoparietal cortex to lateral ventricle; bright subependymal nodules (two marked by curved arrows), located around atrium and temporal horn; and parietal hyperintense linear white matter anomalies (long arrow).
B, Axial SE T1-weighted MR image (500/15/2) shows transmantle dysplasia (short arrows) underlying asymmetrically large perisylvian subarachnoid space; hyperintense probable SGCA (>12 mm) is seen at right foramen of Monro (curved arrow) and subependymal nodules at left foramen of Monro and atrium (only atrium marked, long arrow).
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FIG 3. Infant, imaged at ages 10 weeks, 4 months, and 8 months.
A, Axial SE T1-weighted MR image (500/13/2) at 10 weeks: pachygyric left frontoparietal cortical tuber is less hyperintense than semilunar deep white matter "rim" of transmantle dysplasia (short arrows), which extends all the way to the wall of the lateral ventricle; overlying subarachnoid space is enlarged; various linear white matter anomalies (only two marked by long arrows) are present in both hemispheres; and small subependymal nodules are seen along cellae mediae of both lateral ventricles (not marked).
B, Axial CT scan at 4 months: periphery of transmantle dysplasia is densely calcified, strands of "sunburst" pattern extend toward cortex and left lateral ventricle (not marked); left parietal white matter anomalies (long arrow) are faintly hyperdense; and small subependymal nodules can be seen, for example, in the right lateral ventricle (curved arrow).
C, Axial SE T1-weighted MR image (500/12/2) at 8 months: note that intensity pattern of transmantle dysplasia (not marked) has changed (compare with A); subependymal nodules are visible at cella media of left ventricle (not marked), but white matter anomalies are not visible.
D, Axial high-resolution (surface coil) FSE T2-weighted MR image (4000/112eff/1) at 8 months: transmantle dysplasia is depicted as layered swirls of different signal intensity (arrows).
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FIG 4. Neonate, 2 days (33 weeks' gestation). Axial SE T1-weighted MR image (750/20/2) shows large presumed SGCA at left foramen of Monro (solid arrow), smaller one on right (not marked); hydrocephalus, more pronounced on left; and three cysts adjacent to frontal horns of lateral ventricles (open arrows)
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FIG 5. Infant, 6 months: T1 reversal point.
A, Axial SE T1-weighted MR image (350/20/2) shows two barely distinguishable subependymal nodules (arrow points to more frontal one) along wall of left lateral ventricle; no tuber or white matter anomalies can be seen.
B, Axial CT scan shows hyperdense left frontal wedge-shaped white matter anomalies (arrow).
C, Coronal SE T2-weighted MR image (4000/90/1) shows left frontal tuber and white matter anomalies as slightly hyperintense (arrows).
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FIG 6. Infant, 8 months: "adult" signal pattern.
A, Axial SE T1-weighted MR image (550/20/2) shows multiple bihemispheric tubers (only left frontal and parietal ones are marked by short arrows) and left parietal white matter anomalies (long arrow), all moderately to markedly hypointense; subependymal nodules at both foramina of Monro (only smaller one on right is marked by curved arrow) are moderately hyperintense.
B, Axial SE T1-weighted MR image (550/20/2) shows numerous hypointense tubers (not marked) in both hemispheres.
C, Axial SE T2-weighted MR image (3600/90/1) shows tubers (not marked) as marked hyperintensities.
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