What Is Cerebral Vasculitis?

Cerebral vasculitis, also known as central nervous system (CNS) vasculitis, is a rare disease that causes inflammation or swelling of the blood vessel walls in the brain and spine. Blood vessels are the hollow tubes that transport blood throughout the body. CNS vasculitis can impact the small (capillaries), medium (arterioles and venules), or large (arteries and veins) blood vessels. CNS vasculitis can cause the areas where blood flow has been reduced or stopped to begin to die.

CNS vasculitis is a serious condition. If the blood vessel walls are inflamed, it can stop oxygen from flowing to the brain, which impairs brain functioning and can lead to a stroke. Although this condition is serious and can be life-threatening, it is also treatable.

Cerebral vasculitis can occur with other conditions. Specific conditions include:

• Autoimmune disorders (systemic lupus erythematosus, dermatomyositis, and rarely, sarcoidosis and rheumatoid arthritis)
• Infection (viral or bacterial)
• Systemic vasculitic disorders (granulomatosis with polyangiitis GPA, eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis, cryoglobulinemic vasculitis and Behcet’s syndrome)

When cerebral vasculitis occurs without an associated systemic disorder and is confined to the brain and spine, it is known as primary angiitis of the CNS (PACNS). PACNS is more common in males than females and usually occurs between 40-60 years of age, although it can sometimes occur in children and older adults.

Signs and Symptoms

There are several signs and symptoms of CNS vasculitis. Symptoms will vary among individuals and include:

• New, severe headaches
• Skin rashes
• Seizures
• Fatigue
• Fever
• Weakness
• Unexplained weight loss
• Joint pain
• Abdominal pain
• Forgetfulness or confusion
• Balance or gait issues
• Vision problems
• Strokes or transient ischemic attacks (mini strokes)
• Encephalopathy (swelling of the brain), which can lead to mood and behavioral changes and a decreased level of consciousness
• Kidney issues (dark or bloody urine)
• Nerve issues (pain, numbness, weakness

Causes

Cerebral (or CNS) vasculitis happens when the blood vessels in the brain or spine become inflamed. The exact cause for this inflammation is not always clear. In some vasculitic diseases, autoantibodies attack white blood cells, which then attack the blood vessel walls and cause inflammation and destruction of the vessel walls. Additionally, viruses that cause infection can also lead to developing CNS vasculitis.

Risk Factors

There are specific conditions that CNS vasculitis can occur with. These conditions put people at a greater risk of developing CNS vasculitis. The conditions include:

• Autoimmune disorders (systemic lupus erythematosus, dermatomyositis, and rarely, sarcoidosis and rheumatoid arthritis)
• Infection (viral or bacterial)
• Systemic vasculitic disorders (granulomatosis with polyangiitis GPA, eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis, cryoglobulinemic vasculitis and Behcet’s syndrome) 

When cerebral vasculitis occurs without an associated systemic disorder and is confined to the brain and spine, it is known as primary angiitis of the CNS (PACNS). PACNS is more common in males than females and usually occurs between 40-60 years of age, although it can sometimes occur in children and older adults.

Diagnosis

There are several methods for diagnosing CNS vasculitis. Initially, your doctor will gather information about your symptoms, take a thorough medical history, and perform a physical examination. If vasculitis is suspected, your doctor will order specific bloodwork to assess for any abnormalities. Blood abnormalities include:

• Anemia (deficiency of red blood cells)
• High white blood cell count
• High platelet count (allows the blood to clot)
• Liver or kidney problems
• Elevation of inflammatory biomarkers
• Blood markers that are present in certain types of vasculitis 

Other tests that may be recommended include:

• X-rays
• Tissue biopsies
• Blood vessel scans
• Lumbar puncture (spinal tap)
• Imaging tests (MRI, CT, or angiogram)
• Brain biopsy 

Treatment

CNS vasculitis is typically treated with high-dose corticosteroids in various stages. Corticosteroids help to reduce the inflammation caused by vasculitis. Because CNS vasculitis occurs when the immune system attacks healthy cells, immune suppressant medications are often used in combination with corticosteroids. Treatment is typically more aggressive for the first 6 months and then will taper off over time. Relapse happens in about 25% of the people with CNS vasculitis, and their treatment will be lifelong.

Additionally, for other symptoms of vasculitis that impact movement or memory, other treatment options, such as physical, occupational, or speech therapy may be recommended.

Prevention

There is no known prevention for CNS vasculitis.

Complications

If diagnosis and treatment for CNS vasculitis is significantly delayed, it may lead to permanent neurological issues. Additionally, medication used in treating vasculitis may create complications. Immunosuppressant drugs can make it harder for the body to fight off infections. Usage may also lead to osteoporosis (bone loss). While on these types of medications, regular health monitoring from your doctor is recommended.