Brain Involvement, Seizures, and Stroke-like Episodes

In Sturge-Weber syndrome (SWS), the brain may be affected by a condition called leptomeningeal angiomatosis. This is an abnormal “mat” of blood vessels that forms on the surface of the brain, most often on the same side as the facial birthmark ^[1].

These abnormal vessels do not drain blood efficiently, leading to venous congestion—a backup of blood that prevents oxygen-rich blood from reaching the brain tissue ^[2]. Over time, this lack of oxygen can lead to:

• Cortical Atrophy: A shrinking of the brain tissue in the affected area ^[3].
• Calcifications: Hard mineral deposits that often look like “tram tracks” on imaging. These are markers of past injury from low oxygen levels ^[4].
• Epileptogenic Zone: The injured area of the brain becomes highly sensitive and prone to sending out the irregular electrical signals that cause seizures ^[5].

Seizures: The Most Common Neurologic Sign

Seizures occur in the majority of children with brain involvement in SWS, often starting before their first birthday ^[6][7]. Most of these are focal seizures, meaning they start in one specific part of the brain ^[7].

• What they look like: Seizures may involve rhythmic twitching of one side of the body, staring spells, or unusual eye movements.
• Individualized Seizure Action Plan: Children who have seizures, are at meaningful seizure risk, or are prescribed rescue medicine should have a written plan from their neurologist. This plan explains what to do during a seizure, when to give “rescue” medication at home, and when to call emergency services ^[8]. (Note: If your child has never had a seizure and is low risk, they may not need rescue medication, but a plan for observation is still valuable.)
• Basic Seizure First Aid: Time the event. Protect the child from injury by moving hard objects away. Place them on their side when possible to keep the airway clear. Do not restrain them or put anything in their mouth. Follow your child’s prescribed rescue plan.
• Medications: Doctors typically start with antiseizure medications (ASMs). While many different drugs are used, some studies suggest that medications like oxcarbazepine or carbamazepine may be effective choices for the focal seizures seen in SWS ^[9].

Stroke-like Episodes

A “stroke-like episode” is a hallmark of SWS that can be very frightening for parents. During these events, a child may experience sudden weakness or loss of movement on one side of their body (hemiparesis), vision changes, or prolonged confusion ^[10].

Unlike a typical stroke caused by a blood clot, these episodes in SWS are often related to temporary changes in blood flow, seizures, or a “postictal” (after-seizure) state known as Todd’s paralysis ^[11].

• Urgent Evaluation: Recovery time is highly variable, and a new focal weakness, vision change, severe headache, or prolonged altered awareness needs urgent medical assessment ^[12]. Do not wait for a familiar episode to improve, as distinguishing between a postictal state and a true stroke or neurologic emergency is the doctor’s job ^[13].
• Low-Dose Aspirin: Some specialists use daily low-dose aspirin to help reduce the frequency or severity of these stroke-like episodes ^[14]. Important Safety Warning: Aspirin use in children with SWS is specialist-directed based on observational evidence. Families must never start, stop, or adjust aspirin independently. Discuss bleeding risks, medication interactions, and illness-related risks (such as Reye syndrome during viral infections), as well as protocols for dental or surgical procedures ^[15].

When Medications Aren’t Enough: Epilepsy Surgery

For some children, seizures continue despite trying multiple appropriate and tolerated medications. This is known as drug-resistant epilepsy ^[16]. In these cases, your neurologist should refer your child to a comprehensive epilepsy center for evaluation for epilepsy surgery, such as a hemispherotomy (disconnecting the affected side of the brain) or a focal resection (removing the injured area) ^[17][18].

The goal of surgery is to stop the seizures and protect the healthy parts of the brain, which can help a child’s development and learning ^[19]. In specialized centers and well-selected patients, surgery can lead to high rates of seizure freedom (sometimes 70% to 86%), but results depend on the extent of brain involvement and the center’s experience ^[20]. It is not a guarantee, and surgery comes with important trade-offs such as permanent motor weakness, visual-field deficits, cognitive effects, and rehabilitation needs.

Neurological Red Flags

It is vital to know when a neurological symptom requires immediate medical attention. Call emergency services or go to the nearest emergency room if you notice:

• Status Epilepticus: A seizure that lasts longer than 5 minutes or back-to-back seizures where the child doesn’t wake up in between ^[8].
• Sudden, Severe Weakness: A first, new, or significantly worse loss of movement on one side of the body.
• Extreme Lethargy: Difficulty waking your child or unusual confusion that lasts well beyond the typical “post-seizure” sleepiness.
• Severe, New Headache: Especially if accompanied by repeated vomiting or vision loss.