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

## TL;DR

This paper presents a rare case of Sturge-Weber syndrome type III diagnosed through brain imaging due to the absence of typical skin or eye symptoms.

## Contribution

The study emphasizes the importance of neuroimaging in diagnosing SWS-III when external clinical signs are missing.

## Key findings

- Brain imaging showed cerebral hemihypotrophy, cortical calcifications, and leptomeningeal enhancement in a patient with drug-resistant epilepsy.
- The diagnosis of SWS-III was made without cutaneous or ophthalmological features, relying solely on neuroimaging.
- Accurate diagnosis of SWS-III requires recognition of specific radiological patterns in the absence of external markers.

## 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.