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What is cerebral proliferative angiopathy (CPA)?

Cerebral proliferative angiopathy (CPA) is a rare condition where small, abnormal blood vessels grow throughout the brain and mix with normal brain tissue. These vessels can affect blood flow and may lead to symptoms such as headaches, seizures, or other neurological changes. Because CPA involves both normal and abnormal brain tissue, managing it requires careful monitoring and care as a child grows. One particular challenge is differentiating it from a high-grade brain arteriovenous malformation (AVM), which can often look similar but may carry a different set of risks for a child.

Symptoms & Causes

What are the symptoms of CPA?

Symptoms of CPA can vary and may develop gradually or suddenly in a child. Common symptoms include:

  • Headaches
  • Seizures
  • Stroke-like symptoms such as weakness, numbness, or trouble moving or speaking

What causes CPA?

The exact cause of CPA isn’t fully understood, but it’s thought to result from abnormal blood vessel growth in the brain, sometimes triggered by reduced blood flow. These abnormal vessels can form among normal brain tissue, which affects how the condition progresses and how it’s treated.

Diagnosis & Treatments

How is CPA diagnosed in children?

At Boston Children’s Hospital, we use specialized imaging to accurately diagnose CPA and to create a personalized care plan for each patient. Our pediatric neuroradiologists, neurointerventionists, and neurosurgeons use advanced testing to carefully evaluate the brain and its blood vessels. Because of the complexity of this condition and its similarities to brain AVM, patients may sometimes receive differing diagnoses when evaluated at different centers. Unfortunately, there isn’t a specific blood test, checklist of symptoms, or imaging characteristics that can clearly tell us whether a patient has CPA or a brain AVM. Instead, doctors rely on careful review of advanced imaging and testing — along with experience from similar past cases — to help distinguish between the two.

Advanced testing may include:

  • Catheter angiography to see highly detailed images of abnormal blood vessels
  • MRI to look at the structure of your child’s brain
  • Magnetic resonance angiogram (MRA) or computed tomography angiography (CTA) to see how the blood vessels in the brain are formed and connected
  • Magnetic resonance perfusion (MRP) to see how the blood flow supplies different regions of the brain or the vessel abnormalities
  • Cerebrovascular reserve (CVR) to see how the blood flow to the brain tissue adapts to different demands

How is CPA treated in children?

Treatment for CPA depends on your child’s symptoms and the location of the abnormal blood vessels in the brain. Unlike an AVM, which forms a single tangle of vessels, CPA spreads throughout the brain and mixes with normal brain tissue, making treatment more complex.

At Boston Children’s, members of our Cerebrovascular Surgery and Interventions Center team use their expertise in CPA to choose the safest, most effective approach. Care may include regular monitoring, medications for symptoms like headaches or seizures, and specialized procedures when needed. Surgery is rarely recommended and is used only when needed.

What is the long-term outlook for CPA?

Many children with CPA grow up with symptoms that are mild or well-managed. Some children may need ongoing support for headaches, seizures, or developmental challenges. Regular follow-up with pediatric specialists helps ensure any changes are addressed quickly and care is tailored to each child’s needs.

How we care for cerebral proliferative angiopathy at Boston Children’s Hospital

The Cerebrovascular Surgery and Interventions Center at Boston Children’s is one of the only centers in the world with experience in CPA in children. Our team brings together specialists in neurosurgery, neurology, interventional radiology, neurogenetics, neuropsychology, and other fields to provide comprehensive, coordinated care. This collaborative approach ensures that each patient receives careful monitoring, proactive and personalized symptom management, and access to the most advanced treatment options available. We’re also actively involved in research to better understand CPA and develop approaches that improve outcomes.