Late-onset epilepsy in a surgically-treated Sturge-Weber patient

Sturge-Weber syndrome is a rare, congenital, neurocutaneous disorder. It can be associated with a variety of symptoms including severe epilepsy. Patients often become symptomatic during childhood and the severity of the epilepsy correlates with the patient's neurological outcome. The patient reported here remained asymptomatic until age 24, when he started to experience migraine accompanied by visual scotoma and hemiparesis. Ten years later, he developed severe, refractory epilepsy, with prolonged postictal neurological deficits and ictal as well as post-ictal headaches. It became increasingly difficult to distinguish between migraines and seizures as both could last for several hours, as demonstrated in the two video EEGs. Both the epilepsy and the migraine may therefore have contributed to the patient's severe, neurological deterioration, probably by accelerating the progressive neuronal damage. Surgery improved the situation, but lesional areas were too extensive for complete removal. Late-onset symptoms in Sturge-Weber syndrome may thus result in a severe course for the disease. Early intervention and surgical treatment options are discussed.