Vasculitis of the central nervous system (CNS) is the inflammation of blood vessels in the brain and spinal cord. Surgical treatment for vasculitis may be necessary when vasculitis causes an aneurysm to develop. Aneurysm treatment may include clipping, coiling, or flow diversion.
In vasculitis, the immune system misidentifies parts of blood vessel walls as harmful and attacks the vessel walls, causing them to become swollen and inflamed.
Vasculitis may trigger stroke in one or both of the following ways:
- Ischemic stroke may occur if blood vessel walls become so inflamed and swollen that they block blood flow.
- Hemorrhagic stroke may occur if the vasculitis produces an aneurysm that ruptures.
Vasculitis found only in the CNS, and not associated with any other systemic condition, is called Primary Angiitis of the CNS (PACNS).
Vasculitis anywhere in the body is usually accompanied by systemic symptoms like fever, involuntary weight loss, and/or fatigue.
Symptoms that may accompany vasculitis in the brain include severe headaches, stroke-like symptoms, difficulty with coordination, confusion, or other changes in mental status.
Vasculitis of the spine may cause shooting pains in the arms and legs, numbness, and asymmetrical weakness.
In some cases, the disease may be acute for a time, and then enter periods of remission. In other cases, vasculitis may be chronic.
Early detection and treatment can be of great help to the patient with vasculitis.
Doctors may perform several tests to discover the cause of a patient’s symptoms:
- Blood tests: Vasculitis may cause changes in the blood such as anemia; an increase in the number of white blood cells; and an increase in levels of substances involved with immune and inflammatory reactions.
- Examination of the cerebrospinal fluid: The fluid is taken through a spinal tap (sometimes called a lumbar puncture) and examined for markers of vasculitis.
- Magnetic resonance imaging (MRI): A large magnet and radio waves are used to produce images of the body, including of soft tissues like the brain, spinal cord, and blood vessels.
- Cerebral angiography: A specialist injects dye into the arteries, which makes them visible on X-ray and then takes a series of X-rays to study the flow of blood.
A definitive diagnosis of CNS vasculitis is only possible with a biopsy. To perform a biopsy, a surgeon removes a small amount of tissue from blood vessels in the brain or spine. The biopsy sample is then studied in the laboratory.
Vasculitis is caused by an overactive immune system that mistakenly attacks blood vessel walls. Research into what precipitates the disorder is ongoing.
Sometimes vasculitis is associated with other autoimmune conditions, such as rheumatoid arthritis or lupus. In other cases, an infection or medication can trigger the immune system’s response. Blood vessel disorders that affect the entire body, such as Wegener’s granulomatosis or Behcet’s syndrome, can cause CNS vasculitis.
Fortunately, treatments for vasculitis are helpful, especially in the acute phase. Long-term therapy is a challenge because of side effects of the high-dose steroids or chemotherapeutic agents typically used to treat the disorder.
Treatment may also include the removal and reinfusion of blood plasma or the use of donated blood products.
Surgical treatment focuses on preventing any aneurysms from bursting. Aneurysm treatments include:
- Clipping: Placing small clips onto the blood vessels to close off the blood flow through the aneurysm
- Coiling: Filling the aneurysm with small metal coils that block blood flow
- Flow diversion: Placing a flow diverter, a small tube that directs blood flow through the vessel and away from the aneurysm