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[Magnetic resonance++ with gadolinium in Sturge Weber syndrome]
J Campistol Plana1, J López Castillo, A Capdevila Cirera
1Servicio de Neurología, Hospital Sant Joan de Déu, Barcelona.
Abstract:
We report four patients with Sturge Weber syndrome in which the pial angioma was visualized better with Gd-DOTA enhanced MR imaging. MR alone demonstrated thickened cortex with diminished convolutions, abnormal white matter and prominent subependymal veins. However, Gd-DOTA enhanced MR imaging improved the visualization of the angioma, localizing and demonstrating the extent of the malformation in Sturge Weber syndrome. We believe that this is the procedure of choice to recognize the extent of the intracranial involvement and which would help to select those patients that would benefit from surgical treatment.
Insights
Gadolinium-DOTA enhanced MRI significantly improves visualization of pial angiomas in Sturge Weber syndrome patients. This advanced imaging helps precisely map the malformation
Area of Science:
- Neuroradiology
- Neuroimaging
- Medical Diagnostics
Background:
- Sturge Weber syndrome is a rare congenital disorder characterized by a facial birthmark and neurological abnormalities.
- Intracranialявления of Sturge Weber syndrome include pial angiomas, cortical dysplasia, and leptomeningeal enhancement.
- Accurate assessment of the extent of intracranial involvement is crucial for patient management and surgical planning.
Purpose of the Study:
- To evaluate the efficacy of Gadolinium-DOTA enhanced Magnetic Resonance (MR) imaging in visualizing pial angiomas in Sturge Weber syndrome.
- To compare the diagnostic capabilities of standard MR imaging versus Gd-DOTA enhanced MR imaging for intracranial malformations in Sturge Weber syndrome.
- To determine if enhanced MR imaging can better delineate the extent of the angioma and aid in surgical decision-making.
Main Methods:
- Retrospective analysis of four patients diagnosed with Sturge Weber syndrome.
- Review of MR imaging studies, including standard sequences and those performed with Gadolinium-DOTA contrast enhancement.
- Detailed comparison of lesion visualization, localization, and extent assessment between non-enhanced and enhanced MR images.
Main Results:
- Standard MR imaging revealed thickened cortex, diminished convolutions, abnormal white matter, and prominent subependymal veins.
- Gd-DOTA enhanced MR imaging significantly improved the visualization of the pial angioma.
- Enhanced imaging provided better localization and demonstrated the full extent of the intracranial malformation in all four patients.
Conclusions:
- Gd-DOTA enhanced MR imaging is superior to standard MR imaging for visualizing and assessing the extent of pial angiomas in Sturge Weber syndrome.
- This enhanced imaging technique is valuable for precise diagnosis and surgical planning in affected patients.
- Enhanced MR imaging is recommended as the imaging procedure of choice for evaluating intracranial involvement in Sturge Weber syndrome.