Skip to main content

Sturge Weber Syndrome

Sturge Weber Syndrome (SWS) is a very rare condition whereby a birthmark (port wine stain) is present on the face and there can be an abnormal blood vessels in the brain which lead to seizures. The blood vessels affected in the brain are usually in the occipital lobe at the back of the brain but may be in other parts of the brain too. These abnormalities are present at birth.


The most obvious sign will be a red birthmark which is visible at birth or soon after. It’s usually seen on one side of the face but occasionally is seen on both or on other parts of the body. Two thirds of children affected will have seizures which may begin early in life and are typically focal in presentation.

Focal seizures can cause twitching and jerking of the arm, leg or face on one side and these may spread to involve the whole body (generalised). Other seizure types such as atonic or drop attacks, myoclonic or infantile spasms may be seen. Frequent seizures may lead to a temporary weakness in one side of the body afterwards (hemipareris). With age this weakness may persist. Seizures which become prolonged or which recur without recovery can become status epilepticus which is a medical emergency.

About two thirds of children will have learning difficulties and this may be mild to severe and severity can be associated with greater frequency or duration of seizures.

Visual problems may affect those with SWS due to hemianopia in which the peripheral vision on the weak side is affected. Abnormal blood vessels in the eye can lead to increased pressure or glaucoma which needs treatment to prevent loss of vision.


Where seizures are more prolonged or frequent then learning difficulties may be more pronounced. Children with SWS who have good seizure control tend to have better prognoses. As well as follow up with a paediatric neurologist children with SWS need long term follow up with other specialists to monitor any effects on vision and skin. Long term the condition can be progressive due to effects on blood flow to the part of the brain with the abnormal blood vessels and the prognosis depends on how much of the brain is involved.


The initial presentation of the red birthmark is often the first indication of a potential SWS diagnosis unless it is faint or concealed. A CT brain scan will help identify the abnormal blood vessels on the brain that are characteristic of SWS.


Anti-Seizure Medications

Treatment is mainly with Anti-Seizure Medications (ASM’s) and the aim of treatment is to reduce or control as much as possible as the overall outlook for the child will depend on this. Most ASM’s can be used In SWS and these include Topiramate (Topamax), Carbamazepine (Tegretol), Lamotrigine (Lamictal), Levetiracetam (Keppra), and Sodium Valproate (Epilim).


If seizures persist then epilepsy surgery called a hemispherotomy may be recommended to disconnect the area of the brain with the abnormal blood vessels.

Further Information