Cerebral Arteriopathies in Children

Cerebral arteriopathies are disorders that affect the arteries in the brain. There are several different types of these blood vessel abnormalities, including moyamoya disease, arterial dissection, and vasculitis. About a quarter of cerebral arteriopathies have no known cause. Cerebral arteriopathies are can be found especially in children who have an arterial ischemic stroke (AIS). According to some estimates, about two-thirds of children who experience an AIS have some type of underlying cerebral arteriopathy.

The symptoms of cerebral arteriopathies depend on the specific type of blood vessel abnormality, as well as the particular process affecting the arteries of the brain, but can include severe headaches. If the cerebral arteriopathy causes a blood clot that interrupts blood flow, your child may experience symptoms of an arterial ischemic stroke, such as:

• Weakness on one side of the body
• Difficulty speaking
• Difficulty walking or instability when standing
• Vision loss
• Seizures
• A change in mental state

Short episodes of weakness or numbness on one side of the body, difficulty speaking, or a sensation that the room is spinning may mean your child is having a transient ischemic attack, a possible warning sign of stroke that requires immediate evaluation in an emergency room.

However, in inflammatory arteriopathy that affects the brain, a child may also experience:

• Headache
• Decline in cognitive function
• Change in personality
• Increased sleepiness
• Seizure

About one-quarter of cerebral arteriopathies in children have no known cause. Other types of cerebral arteriopathies, such as moyamoya disease, appear to have a genetic component. Arterial dissection is often the result of an injury or other trauma to a child's head or neck. Finally, inflammation and infection are now being increasingly associated with vascular inflammation in the brain and can lead to stroke in childhood.

Children with sickle cell disease appear to be at higher risk for developing cerebral arteriopathies.

If a physician suspects your child has a cerebral arteriopathy, they may use a number of imaging tests to get detailed images of your child's blood vessels and brain tissue. Tests can include:

• CT angiography (CTA), which uses X-ray equipment and powerful computers to create cross-sectional images (often called "slices") of the head, neck, and brain. CTA uses a special dye (known as contrast) that is injected into a vein. A computer generates detailed 3D images of the blood vessels.
• Magnetic resonance angiography (MRA), which visualizes blood vessels in the brain, head, and neck, but without the need for X-rays. Unlike CTA, many MRA scans can be done without injecting a dye into the veins to generate images of the vessels. The Stroke and Cerebrovascular Center also collaborates with the Division of Neuroradiology on special MRI vascular imaging scans that look for evidence of inflammation in the vessel walls of brain arteries. If found, this can then be treated.
• Catheter angiography, which is done under general anesthesia. A catheter (a flexible tube about the width of spaghetti) is placed directly into the child's arteries. The catheter is used to inject a special dye, known as contrast, allowing us to get more detailed images.

Treatment depends on the specific type of cerebral arteriopathy your child has but can include:

• Medications to help prevent blood clots
• Minimally invasive surgical techniques to remove clots or repair blood vessel abnormalities
• Medicines to treat inflammation and infection
• Rehabilitation if your child has experienced a stroke

Children who have experienced a stroke as the result of a cerebral arteriopathy receive treatment from the team of experts in our Stroke and Cerebrovascular Center. Treatment may include the use of drugs to stop inflammation or to decrease blood clotting. When surgery is necessary to treat the underlying arteriopathy, we work with our colleagues in the Cerebrovascular Surgery and Interventions Center to coordinate seamless interdisciplinary care of these children.