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Case Reports
. 2025 Mar 9;20(5):2487-2490.
doi: 10.1016/j.radcr.2025.01.092. eCollection 2025 May.

Isolated leptomeningeal angiomatosis in Sturge-weber syndrome type III: A case report with distinctive neuroimaging features

Azzeddine Laaraje  1 , Salah Ben Elhend  2
Affiliations
Case Reports

Isolated leptomeningeal angiomatosis in Sturge-weber syndrome type III: A case report with distinctive neuroimaging features

Azzeddine Laaraje et al. Radiol Case Rep. .

Abstract

Sturge-Weber syndrome type III (SWS-III) is the rarest variant of SWS, characterized by isolated leptomeningeal angiomatosis without cutaneous or ophthalmological manifestations. We report the case of an 11-year-old female who developed drug-resistant epilepsy at 18 months of age and mild left hemiparesis. Despite multiple anticonvulsant medications, seizures remained poorly controlled. Brain imaging revealed characteristic findings of right cerebral hemihypotrophy, cortical calcifications, and leptomeningeal enhancement without facial port-wine stain or ophthalmological involvement. These distinctive radiological features led to the diagnosis of SWS-III. This case highlights the crucial role of neuroimaging in diagnosing SWS-III, where external clinical markers are absent. Recognition of specific radiological patterns is essential for accurate diagnosis and appropriate management.

Keywords: Cortical calcifications; Drug-resistant epilepsy; Leptomeningeal angiomatosis; Magnetic resonance imaging; Neuroimaging; Sturge-Weber syndrome type III.

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Figures

Fig. 1 –
Fig. 1
Axial noncontrast brain CT in Sturge-Weber syndrome type III The image shows right cerebral hemihypotrophy with spontaneous cortical hyperdensities corresponding to fine calcifications (*). These gyriform calcifications are characteristic of SWS and represent chronic hypoxic injury to the cortex.
Fig. 2 –
Fig. 2
Coronal FLAIR MRI in Sturge-Weber syndrome type III. The image demonstrates hemiatrophy of the right cerebral hemisphere with prominent involvement of temporal and parietal lobes (black arrow), associated with congested veins (white arrow). These findings reflect chronic hypoperfusion and venous congestion typical of SWS.
Fig. 3 –
Fig. 3
Axial T2* MRI in Sturge-Weber syndrome type III The sequence reveals dilatation of transparenchymal veins (*) with enlarged ipsilateral choroid plexus (black arrow) and calvarial thickening (white arrow). These features represent the characteristic parenchymal pattern of SWS type III.
See this image and copyright information in PMC

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