Sturge Weber Syndrome is a congenital disorder characterized by facial capillary malformations and capillary-venous malformations affecting the brain and eye. The capillary-venous malformations can occur in the leptomeninges, choroid, or conjunctiva, creating an epileptogenic focus, glaucoma, hemiparesis or visual field defects.

We report a patient who, at the age of 54, presented with headache, right sided visual field deficit, progressive encephalopathy and ultimately new-onset seizures. His past medical history was significant for hypothyroidism and glaucoma OS. Physical exam revealed a violaceous rash over the left eyelid and forehead. Neurologic exam showed no focal deficits. MRI Brain with and without contrast revealed abnormal T2/FLAIR hyperintensity in the left parietal, temporal and occipital lobes along with the leptomeninges in the same region.  The left choroid plexus avidly enhanced and there was left hemiatrophy. The patient was diagnosed with Sturge-Weber Syndrome from the clinical features and characteristic MRI findings. 

If present, seizures typically present within the first 5 years of life in patients with Sturge Weber Syndrome. However, this patient presented at the age of 54. These cases are atypical and often misdiagnosed as hemiplegic migraine.

This patient is one of the oldest reported with new-onset seizures as the first manifestation of Sturge-Weber Syndrome. Violaceous facial rashes and seizures should always bring Sturge-Weber Syndrome into the differential diagnosis. Co-morbid seizures may occasionally present late in life and due to their potential origination from the occipital lobes, may present with features similar to migraine headache with visual aura.