What is Central Nervous System Vasculitis?

Central nervous system (CNS) vasculitis is inflammation of blood vessel walls in the brain or spine. (The brain and the spine make up the central nervous system.) CNS vasculitis often occurs in the following situations:

•  accompanied by other autoimmune diseases such as systemic lupus erythematosus, dermatomyositis, and, rarely, rheumatoid arthritis

• infection, such as viral or bacterial

• systemic (affecting the whole body) vasculitic disorders (Wegener’s granulomatosis, microscopic polyangiitis, Behçet’s syndrome);

• it can be without any associated systemic disorder. In this case, the vasculitis is only confined to the brain or the spinal cord; and, it is referred to as primary angiitis of the CNS (PACNS).

What is the cause of Central Nervous System Vasculitis?

How the vessels in the brain become inflamed is not entirely clear. In some diseases, abnormal antibodies (autoantibodies) and white blood cells attack vessel walls and cause inflammation and destruction of the vessel wall. Infection caused by a virus can also cause CNS vasculitis.

Is Central Nervous System Vasculitis dangerous?

CNS vasculitis can be a serious condition. The inflamed vessel wall can block the flow of oxygen to the brain, causing a loss of brain function. In some cases, CNS vasculitis is life-threatening.

Symptoms

Symptoms of CNS vasculitis can include the following:

• severe headaches that last a long time
• strokes or transient ischemic attacks (“mini-strokes”)

• forgetfulness or confusion
• weakness

• problems with eyesight
• seizures
• encephalopathy (swelling and damage to the brain)
• sensation abnormalities

Diagnosis

The diagnosis of vasculitis, including CNS vasculitis, is based on a person’s medical history, symptoms, a complete physical examination, and the results of special laboratory tests. Blood abnormalities that are found in vasculitis include:

• anemia (low red blood cell count)
• high white blood cell count
• high platelet count
• kidney or liver problems
• allergic reactions
• finding immune complexes in the circulating blood
• identifying abnormal antibodies in the blood
• an increase in blood markers of inflammation