Central Nervous System (CNS) Vasculitis

Central nervous system (CNS) vasculitis is a rare type of inflammation that affects the blood vessels of the brain, spinal cord, or both. It is an autoimmune disorder, which occurs when the body’s immune system attacks healthy tissues. CNS vasculitis can affect both children and adults.

There are two forms of CNS vasculitis:

• Primary CNS vasculitis only affects the brain, spinal cord, or both, and isn’t related to any other systemic disease. 
• Secondary CNS vasculitis usually occurs along with another autoimmune disease (such as lupus) or an infection.

CNS vasculitis can also be categorized based on the size of the blood vessels affected:

• Large vessel CNS vasculitis affects medium to large blood vessels. This can be detected with noninvasive imaging tests such as magnetic resonance or computed tomography angiography (MRA or CTA). Large vessel CNS vasculitis can be further divided into progressive and non-progressive subtypes. Children with large vessel CNS vasculitis typically have acute stroke symptoms, such as weakness, numbness, difficulty speaking, and vision problems.
• Small vessel CNS vasculitis affects the blood vessels that are too small to be seen on MRA or CTA. A brain biopsy is required to make this diagnosis. Children with small vessel CNS vasculitis usually experience a combination of symptoms, including headaches, cognitive/behavioral changes, seizures, and stroke symptoms.

Depending on the type of CNS vasculitis your child has, they may experience symptoms including:

• Headaches
• Difficulties with thinking and memory
• Problems with coordination
• Muscle weakness or paralysis
• Seizures
• Behavioral changes
• Stroke or stroke-like symptoms

Note: If you believe your child is having a seizure or a stroke, please seek immediate medical attention.

The exact cause of primary CNS vasculitis is still unknown. Secondary CNS vasculitis appears to occur in children who have other autoimmune diseases (such as systemic lupus erythematosus, Kawasaki disease, or inflammatory bowel disease). It also appears to occur in response to a bacterial or viral infection.

CNS vasculitis can be challenging to diagnose, especially when there is no other underlying autoimmune disease or infection present. Your child’s doctor will perform a physical and neurological exam and ask about their medical history and symptoms. They may also recommend other tests to help diagnose CNS vasculitis, such as:

• Blood tests
• MRI scan
• CT scan
• Cerebral angiogram
• Brain biopsy

Several other conditions can mimic CNS vasculitis, which can make it difficult to diagnose. In such cases, antibody and genetic testing through blood tests may be able to diagnose a different condition, making a brain biopsy unnecessary. In fact, Boston Children’s Neuroimmunology Center has published a key study showing that as many as two-thirds of brain biopsies looking for small vessel CNS vasculitis may be unnecessary. Our center has extensive expertise in determining which patients do or do not require brain biopsy.

The goal of treatment for CNS vasculitis is to relieve inflammation in the brain and spinal cord, which will then help ease symptoms, helping to prevent strokes. The exact treatment regimen is tailored to the specific subtype of CNS vasculitis. Treatments can include:

• Corticosteroids. Steroid medications help relieve inflammation and address related symptoms. They are typically given intravenously at high doses for a set number of days, then switching to an oral dose, tapering off over several weeks to months.
• Immunosuppressive medications. These include cyclophosphamide, mycophenolate mofetil, infliximab, and rituximab.
• Stroke preventive medications. These include aspirin, clopidogrel, or low molecular weight heparin.
• Other treatments. Your child’s doctor may prescribe other medications, such as headache or anti-seizure drugs, to treat certain symptoms of CNS vasculitis.

At Boston Children’s Hospital, we care for children with central nervous system vasculitis in our Neuroimmunology Center. Our team of pediatric neuroimmunologists takes a multidisciplinary approach, working with other specialists throughout Boston Children’s who have expertise in caring for children with central nervous system vasculitis, including stroke neurologists, rheumatologists, hematologists, neurosurgeons, physical therapists, education specialists, and others. Together, we will create a treatment plan that puts your family in the center.