Central Nervous System Vasculitis | Symptoms & Causes
What are the symptoms of central nervous system vasculitis?
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.
What causes central nervous system vasculitis?
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.
Central Nervous System Vasculitis | Diagnosis & Treatments
How is central nervous system vasculitis diagnosed?
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:
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.
How is central nervous system vasculitis syndrome treated?
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.
How we care for central nervous system vasculitis in children
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.
