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Sturge-Weber syndrome Type I: a rare case report
Batoul Hendieh1, Firas Khana2, Sevin Ibrahim1
1Department of Pediatrics, Faculty of Medicine, University of Aleppo, Aleppo University Hospital (AUH), Aleppo, Syria.
Annals of Medicine and Surgery (2012)
|September 3, 2025
Summary
This case documents a rare instance of Sturge-Weber syndrome (SWS) Type I presenting with acute neurological symptoms. Early diagnosis and treatment led to full recovery, highlighting SWS
Area of Science:
- Neurocutaneous disorders
- Vascular malformations
- Neurology
Background:
- Sturge-Weber syndrome (SWS) is a rare congenital disorder characterized by facial port-wine stains, leptomeningeal angiomas, and ocular abnormalities.
- SWS typically presents with neurological deficits such as seizures and developmental delay, often associated with extensive brain involvement.
Observation:
- An 11-year-old boy with SWS Type I presented with acute neurological symptoms including vomiting, headaches, left-sided hemiparesis, and right-sided facial deviation.
- Despite extensive temporal lobe angiomatosis, the patient initially maintained preserved cognition, deviating from the typical SWS presentation.
- The patient experienced stroke-like episodes attributed to fragile leptomeningeal vasculature.
Findings:
- The patient received treatment with anticonvulsants and low-dose aspirin.
- Full neurological recovery was achieved within 3 months of treatment initiation.
- The patient remained stable during a 2-year follow-up period.
Implications:
- This case highlights the diverse clinical manifestations of Sturge-Weber syndrome.
- Accurate diagnosis through advanced imaging is crucial for effective management.
- Early intervention and tailored treatment are essential for preventing complications and improving patient outcomes in SWS.
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