One Patient with Sturge-Weber Syndrome and Stroke-Like Episodes: Our Experience with Acetylsalicylic Acid

Introduction: The association of cerebral leptomeningeal angioma and ipsilateral facial nevus flammeus in the territory of the first branch of the trigeminal nerve is known as the sporadic neurocutaneous disease Sturge-Weber syndrome (SWS). The cases with the absence of a facial angioma are usually considered to be variants of this syndrome. Among other frequent symptoms such as seizures, headaches, developmental delay, and glaucoma, stroke-like episodes are only sporadically reported associated features. There is no evidence-based therapeutic recommendation for this condition. A few case reports suggest that prophylactic treatment with low-dose acetylsalicylic acid (ASS) may prevent the neurological decline.

Case Report: This almost 11-year-old girl had uneventful early development. She was diagnosed in her 2nd year, after she had her first seizures consisting of right hemiconvulsions. No facial nevus was present at birth. The magnetic resonance imaging demonstrated unilateral brain involvement showing atrophy and calcifications in the left temporo-parieto occipital region, compatible with SWS. The neurological examination revealed no neurological signs. After the administration of valproate, the cessation of seizures was achieved. At the age of 8 years, she developed paroxysmal events with a different semiology. A few times per year she vomited, had moderate headache, and a stiff neck. A sudden onset of mild numbness of the right leg and disturbance in the visual field accompanied these episodes. No other symptoms occurred. We saw one habitual episode soon after the admittance in our center. All symptoms resolved during the following 24 hours. The electroencephalography performed during and after this attack showed no epileptic activity but regional slowing with complete resolution. The etiology of attack was presumed to be ischemic, so-called stoke-like episode and a prophylaxis with ASS (2 mg/kg/d) was initiated. Thereafter, the paroxysmal events stopped and have not recidivated in the past 18 months of follow-up.

Conclusion: Early recognition and energetic therapy of symptoms remains a cornerstone in the management of SWS. ASS is potentially safe and effective prophylaxis of neurological deterioration in individuals with SWS, although its use in the pediatric population remains controversial disputed. In the absence of guidelines and randomized controlled trials, individual case reports increase the knowledge and clinical competence in confronting these patients.