Type 3 Sturge-Weber Syndrome Presenting With Concurrent Epilepsy and Migraine

伴有癫痫和偏头痛的3型Sturge-Weber综合征

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Abstract

Sturge-Weber syndrome is a rare congenital disorder with vascular malformations affecting the brain, skin, and eyes. Type 3 Sturge-Weber syndrome, the rarest form, presents with isolated leptomeningeal angiomas, often causing diagnostic delays. This report highlights an unusual presentation where both recurrent focal seizures and migraine-like headaches were prominent, long-standing features. A 30-year-old man presented with two focal seizures affecting the right side of his body, preceded by aura and followed by postictal confusion, right-sided weakness, and migraine-like headache with photophobia and vomiting. He had experienced focal seizures since childhood, with worsening postictal symptoms and progressive headaches over time. Examination revealed disorientation and right-sided weakness, while laboratory findings showed hyperglycemia, consistent with undiagnosed diabetes. Neuroimaging revealed leptomeningeal enhancement, tram-track calcifications, and cortical atrophy in the left parietal region, confirming type 3 Sturge-Weber syndrome. Notably, the patient had newly diagnosed diabetes, suggesting a possible metabolic link to Sturge-Weber syndrome. Management included antiepileptic therapy and glycemic control, leading to clinical improvement. The heterogeneous neurological presentation of type 3 Sturge-Weber syndrome often delays recognition. Early neuroimaging and a high index of suspicion are essential for timely diagnosis, particularly in the absence of cutaneous features.

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