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Neuroimaging of Tuberous Sclerosis: Spectrum of Pathologic Findings and Frontiers in Imaging

Babak N. Kalantari1 and Noriko Salamon

1 Both authors: Department of Radiology, David Geffen School of Medicine at UCLA, 10833 Le Conte Ave., Los Angeles, CA 90095-1721.


Figure 1
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  		Fig. 1A —4-year-old girl with tuberous sclerosis complex. Axial T1-weighted (A) and T2-weighted (B) MR images show T1 hypointensity (arrows, A) and T2 hyperintensity (arrows, B) in subcortical white matter of left frontal and right parietal lobes. Gray–white matter differentiation is partially obliterated. Findings are characteristic of cortical and subcortical tubers.

 

Figure 2
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  		Fig. 1B —4-year-old girl with tuberous sclerosis complex. Axial T1-weighted (A) and T2-weighted (B) MR images show T1 hypointensity (arrows, A) and T2 hyperintensity (arrows, B) in subcortical white matter of left frontal and right parietal lobes. Gray–white matter differentiation is partially obliterated. Findings are characteristic of cortical and subcortical tubers.

 

Figure 3
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  		Fig. 2A —11-month-old boy with tuberous sclerosis complex. Axial FLAIR MR images show subtle bilateral cortical and subcortical tubers (arrow) in right frontal (A) and left parietal (B) lobes.

 

Figure 4
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  		Fig. 2B —11-month-old boy with tuberous sclerosis complex. Axial FLAIR MR images show subtle bilateral cortical and subcortical tubers (arrow) in right frontal (A) and left parietal (B) lobes.

 

Figure 5
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  		Fig. 3 —7-year-old boy with tuberous sclerosis complex. Axial T1-weighted MR image shows small subependymal nodule (arrow) projecting into lumen of right lateral ventricle.

 

Figure 6
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  		Fig. 4A —4-year-old girl with tuberous sclerosis complex. Axial unenhanced T1-weighted (A) and contrast-enhanced T1-weighted (B) MR images show enhancing subependymal nodule (black arrow) projecting into lumen of left lateral ventricle. Subependymal nodules, which contain more calcification, tend to become less enhanced, as in case of nodule (white arrow) located near left atrium.

 

Figure 7
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  		Fig. 4B —4-year-old girl with tuberous sclerosis complex. Axial unenhanced T1-weighted (A) and contrast-enhanced T1-weighted (B) MR images show enhancing subependymal nodule (black arrow) projecting into lumen of left lateral ventricle. Subependymal nodules, which contain more calcification, tend to become less enhanced, as in case of nodule (white arrow) located near left atrium.

 

Figure 8
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  		Fig. 5A — 9-year-old girl with tuberous sclerosis complex and partial complex seizures. Coronal contrast-enhanced T1-weighted image shows homogeneously enhancing multilobulated subependymal giant cell astrocytoma (black arrow) measuring 238 mm2 in region of left foramen of Monro. Patient has undergone left temporal lobe resection (white arrow).

 

Figure 9
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  		Fig. 5B — 9-year-old girl with tuberous sclerosis complex and partial complex seizures. Coronal contrast-enhanced T1-weighted image obtained 3 months after initiation of oral rapamycin therapy shows size of subependymal giant cell astrocytoma (black arrow) has decreased to 126 mm2. White arrow indicates site of left temporal lobe resection.

 

Figure 10
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  		Fig. 6 —8-year-old boy with tuberous sclerosis complex. Axial FLAIR MR image shows cortical and subcortical tuber (black arrow) and radial migration line (white arrow).

 

Figure 11
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  		Fig. 7A —6-year-old girl with tuberous sclerosis complex. Three-dimensional MRI reconstructions show total cerebral volume of 994 cm3 (A) compared with 1,290 cm3 in age-matched patient without tuberous sclerosis complex (B).

 

Figure 12
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  		Fig. 7B —6-year-old girl with tuberous sclerosis complex. Three-dimensional MRI reconstructions show total cerebral volume of 994 cm3 (A) compared with 1,290 cm3 in age-matched patient without tuberous sclerosis complex (B).

 

Figure 13
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  		Fig. 8A —9-year-old boy with tuberous sclerosis complex. Axial 18F-FDG PET (A) and PET/MRI fusion (B) images show multiple areas of hypometabolism corresponding to tubers (arrows).

 

Figure 14
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  		Fig. 8B —9-year-old boy with tuberous sclerosis complex. Axial 18F-FDG PET (A) and PET/MRI fusion (B) images show multiple areas of hypometabolism corresponding to tubers (arrows).

 

Figure 15
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  		Fig. 9A —10-month-old boy with tuberous sclerosis complex and intractable seizures. Axial FLAIR MR image shows cortical and subcortical tubers (arrows) in both temporal lobes.

 

Figure 16
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  		Fig. 9B —10-month-old boy with tuberous sclerosis complex and intractable seizures. Axial MRI/PET fusion image shows multiple areas of hypometabolism corresponding to tubers (white arrows). Tuber (red arrow) in right anterior temporal lobe shows disproportionately large area of hypometabolism compared with its size in A, indicating zone is probably epileptogenic.

 

Figure 17
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  		Fig. 9C —10-month-old boy with tuberous sclerosis complex and intractable seizures. Axial T2-weighted MR image shows right temporal region after resection of epileptogenic focus (arrow). Patient became seizure-free.

 

Figure 18
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  		Fig. 10A —3-year-old girl with tuberous sclerosis complex and seizures. Axial T2-weighted image shows left cerebellar tuber with typical wedge-shaped configuration (arrow).

 

Figure 19
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  		Fig. 10B —3-year-old girl with tuberous sclerosis complex and seizures. Axial MRI/PET fusion image shows area of hypometabolism approximately equal in size to cerebellar tuber (arrow) in keeping with fact that cerebellar tubers are rarely epileptogenic.

 

Figure 20
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  		Fig. 11 —Photograph shows magnetoencephalography machine. (Courtesy of MEG International Services Ltd.)

 

Figure 21
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  		Fig. 12A —17-year-old boy with tuberous sclerosis complex and intractable seizures. Axial FLAIR MR image shows multiple bilateral cortical and subcortical tubers (arrows).

 

Figure 22
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  		Fig. 12B —17-year-old boy with tuberous sclerosis complex and intractable seizures. Axial PET/MRI fusion image shows multiple areas of hypometabolism (red arrows) corresponding to tubers. Tuber in left posterior temporal lobe shows disproportionate area hypometabolism (orange arrow) compared with size in A, indicating zone is probably epileptogenic.

 

Figure 23
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  		Fig. 12C —17-year-old boy with tuberous sclerosis complex and intractable seizures. Axial T1-weighted magnetic source image shows epileptiform activity (yellow lines) arising from location of tuber that exhibits hypometabolism in B.

 

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