MR imaging of tuberous sclerosis: pathogenesis of this phakomatosis, use of gadopentetate dimeglumine, and literature review.

Abstract

Findings on cranial magnetic resonance (MR) images were correlated with known histopathologic findings in 42 patients with tuberous sclerosis (TS), 17 of whom received gadopentetate dimeglumine, to extend the range of signs on MR images. Four neuroradiologists recorded the number, sites, configurations, and relative signal intensities of intracranial abnormalities. White matter lesions, found in 39 patients (93%), showed four distinct patterns: (a) straight or curvilinear bands extending radially from the ventricle through the cerebral mantle toward the cortex, (b) wedge-shaped lesions, (c) nonspecific conglomerate foci, and (d) cerebellar radial bands. It is concluded that cortical tubers, white matter lesions, subependymal nodules, and sub-ependymal giant cell astrocytomas (SGCAs) may be enhanced after administration of gadopenetetate dimeglumine. Enhancement of a TS lesion does not indicate neoplastic transformation into SGCA. Imaging surveillance every 12 months appears indicated during the peak ages (8-18 years) of occurrence of SGCA. The appearance of white matter lesions supports the theory that the cerebral lesions of TS may be caused by disordered migration of dysgenetic cells.

Cerebral Diffusion Tensor MR Tractography in Tuberous Sclerosis Complex: Correlation with Neurologic Severity and Tract-Based Spatial Statistical Analysis

“…2 On neuroradiologic examination, tuberous sclerosis complex shows cortical tubers, transmantle white matter lesions, subependymal nodules, and/or tumors. 3 Many researchers have studied the relationship between brain MR features and seizures, developmental disability, or autism in patients with tuberous sclerosis complex. [4][5][6][7] A recent study correlated neurologic outcome with cortical tuber burden and transmantle white matter lesions, resulting in a proposed composite clinical scoring system assessing major neurologic features of tuberous sclerosis complex.…”

Ring-shaped Lateral Ventricular Nodules Detected with Brain MR Imaging

“…Subependymal nodules are found in patients with tuberous sclerosis and exhibit a one-to 12-mm diameter, variable signal intensity on MR imaging, and often calciˆca-tion; most demonstrate some enhancement. 8 Reported only once in radiological journals, 1 RSLVNs may be unfamiliar among neurosurgeons and radiologists other than neuroradiologists. If a neurosurgeonˆnds these nodules during ventriculoscopy, they can perform biopsy, but such biopsy has not been reported because of the low prevalence of RSLVNs and their incidental discovery.…”

Surgical timing of the subependymal giant cell astrocytoma (sega) with the patients of tuberous sclerosis complex. .

“…TSC derives its name from cortical tubers, so called for their gross pathologic "potatolike" appearance (9). They occur in as many as 95% of patients (3,17). Tubers most commonly involve surface cortical and…”

“…The classic TSC central nervous system findings of cortical tubers, subependymal nodules (SENs), subependymal giantcell astrocytomas (SEGAs), and white-matter abnormalities are now easily identified by modern neuroimaging techniques (3,4,17,19,20). SEGAs are benign tumors that occur in approximately 10% of patients with TSC.…”