Central Nervous System (CNS) Vasculitis

Central nervous system (CNS) vasculitis is a rare condition in which blood vessels become inflamed, restricting blood flow and causing damage to organs and tissues. It’s believed to be caused by an autoimmune process and can block blood vessels that supply the brain and spinal cord, causing loss of brain function or a stroke.

There are two categories of CNS vasculitis:

• Primary CNS Vasculitis affects the brain and spinal cord only.
• Secondary CNS vasculitis occurs along with other autoimmune diseases such as lupus, dermatomyositis, rheumatoid arthritis and others.

Like other autoimmune disorders, the causes of CNS vasculitis are not fully understood. For some reason, the body starts to attack healthy tissue as if it was unhealthy. Genetic and environmental factors may play a role in the condition.

Symptoms of CNS Vasculitis

• A severe headache that won’t go away
• Forgetfulness or confusion
• Changes in behavior
• Muscle weakness or paralysis
• Lack of coordination
• Abnormal sensations or a loss of sensation
• Problems with vision
• Seizures
• A stroke or transient ischemic attacks

Diagnosing CNS Vasculitis

Diagnosis can be difficult, as CNS vasculitis shares symptoms with several other diseases.  However, if caught early, treatment can be successful at minimizing symptoms. There are several diagnostic options for CNS vasculitis:

• Blood tests
• Spinal tap to examine spinal fluid
• CT scans and MRIs
• Cerebral angiogram
• Biopsy of a blood vessel or affected organ

Treatment

Doctors normally prescribe high-dose steroids, such as prednisone, to bring down inflammation. In more severe cases, this may be combined with drugs that inhibit the body’s immune system.

The medications prescribed to treat CNS vasculitis can have serious side effects, such as lowering your body’s ability to fight infection. Effective treatment requires a coordinated effort between you and your care team. Relapses are not uncommon.