STURGE-WEBER SYNDROME

Seizures

Focal seizures are usually the commonest seizure type, with features referable to the location of the leptomeningeal angioma. If these do not have motor features, they may be difficult to detect in infants.

Focal to bilateral tonic-clonic seizures can occur, these may have focal features referable to the location of the leptomeningeal angioma if this information is carefully sought.

Epileptic spasms may be seen, these may have focal features that are referable to the location of the leptomeningeal angioma.

Status epilepticus, including focal status epilepticus, is seen, and is associated with increased risk of stroke.

CAUTION patients with Sturge-Weber syndrome, who have earlier age of seizure onset, may have a change in their seizure types over time, with the emergence of epileptic spasms or generalized seizure types, such as atypical absence, atonic and tonic seizures. Developmental and cognitive impairments are more common in this group. If these seizure types are seen, achieving full seizure control is important, if this is not achieved with medication, epilepsy surgery should be considered.