Modarage Nadeeka Anuradhi Peiris¹, Ann Modaragama², Darshika Gunawardana ¹, Mihiri Peiris ³, Kapila Panditha ¹, Sanjaya Fernando ¹
1- Lady Ridgeway Hospital for Children, Sri Lanka
2- Colombo North Teaching Hospital, Sri Lanka
3- Teaching Hospital Karapitiya, Sri Lanka
Corresponding Author:
Modarage Nadeeka Anuradhi Peiris (
nadeeka.best@gmail.com)
Abstract Body
Introduction: Sturge-Weber syndrome (SWS) is a sporadic vascular developmental disorder. While classically associated with a facial capillary malformation, some children present without skin signs, delaying diagnosis. There are three types of SWS according to the Roach scale, where type 3 presents with only leptomeninges without neurocutaneous or eye involvement.
Case presentation: A 1-year-7-month-old girl with left focal motor seizures since 1 year of age, poorly responsive to multiple anti-seizure medicines, presented with three episodes of transient left-sided weakness lasting for 2-3 minutes over 48 hours. Previous post-ictal weakness had been treated as Todd’s paralysis at 1 year of age. Her development was age-appropriate. Normal neurological manifestation, no neurocutaneous manifestations. Full blood count, electrolytes, renal and liver functions, arterial blood gas, ammonia, lactate, uric acid and coagulation studies were normal. EEG showed right fronto-parietal epileptiform discharges. MRI brain revealed mild right hemispheric volume loss with abnormal vasculature consistent with SWS (Roach Type III). Ophthalmology assessment was normal.
Conclusions: This case highlights Roach Type III SWS as an important consideration in refractory focal epilepsy with recurrent transient hemiparesis even without a facial capillary malformation. Follow-up arranged with regular ophthalmology screening for glaucoma 3-6 monthly in early childhood, then annually , neurodevelopmental surveillance, optimisation of seizure control, referral to an epilepsy surgery programme if seizures remain medically refractory , and caregiver education on emergency seizure management.