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MR imaging of tuberous sclerosis in neonates and young infants
1Department of Radiology, University of California at San Francisco, 94143, USA.
Insights
Tuberous sclerosis lesions in infants under 3 months appear hyperintense on T1-weighted MR images and hypointense on T2-weighted images. Infant MR imaging facilitates tuberous sclerosis diagnosis, so imaging should not be delayed if suspected.
Area of Science:
- Neurology
- Radiology
- Pediatrics
Background:
- Intracranial manifestations of tuberous sclerosis exhibit age-dependent changes on MR imaging.
- Understanding these changes is crucial for accurate diagnosis in neonates and infants.
Purpose of the Study:
- To characterize the specific MR imaging features of intracranial tuberous sclerosis in neonates and infants.
- To differentiate infantile patterns from those observed in older individuals.
Main Methods:
- Retrospective review of MR studies in seven patients under 3 months old.
- Analysis of lesion appearance, number, and distribution, including tubers, white matter anomalies, and subependymal nodules/tumors.
Main Results:
- Infants showed increased white matter anomalies, subependymal nodules, subependymal giant cell astrocytomas, transmantle dysplasias, and left-hemispheric/temporal lesions.
- Cortical tubers were less prevalent in infants compared to older patients.
- Lesions appeared as hyperintense foci on T1-weighted images and poorly visible hypointensities on T2-weighted images.
Conclusions:
- Infantile tuberous sclerosis lesions are hyperintense on T1 and hypointense on T2-weighted MR images, contrasting with the pattern in older individuals.
- Reduced myelination aids in identifying white matter anomalies in infants.
- Despite challenges with intracortical tubers, the increased conspicuity of other lesions facilitates diagnosis in infants, supporting prompt imaging when suspected.
Background And Purpose:
The MR imaging appearance of intracranial manifestations in tuberous sclerosis varies with age. The aim of this study was to specify MR characteristics in a coherent group of neonates and infants in order to distinguish them from the mature pattern.
Methods:
The MR studies of seven patients under 3 months old were reviewed retrospectively. Imaging appearance, number, and distribution of tubers, white matter anomalies, subependymal nodules, and subependymal giant cell astrocytomas were analyzed.
Results:
All patients had more white matter anomalies, subependymal nodules, subependymal giant cell astrocytomas, transmantle dysplasias, and left-hemispheric and temporal lesions, but less cortical tubers than did older patients in previous series. The lesions were easy to detect as hyperintense foci on T1-weighted images. Visibility as hypointensities on T2-weighted images was comparatively poor.
Conclusion:
The nodular subependymal and linear parenchymal tuberous sclerosis lesions in infants under 3 months old are hyperintense on T1-weighted images and hypointense on T2-weighted images as opposed to the reverse pattern of signal intensity in older persons. The scarce myelination helps to identify white matter anomalies, which become less visible as myelination progresses. Conversely, purely intracortical tubers are more difficult to diagnose in infants. Because the overall number and conspicuity of all other lesions in our series were greater than in previous series with older subjects, our findings indicate that infant age does not compromise, but facilitates, the correct MR diagnosis of tuberous sclerosis. Therefore, if tuberous sclerosis is clinically suspected within the first 3 months of life, imaging should not be delayed.