Download Primary Central Nervous System Vasculitis

Primary Central Nervous System Vasculitis
Education Content:
central nervous system vasculitis
Central nervous system (CNS) vasculitis is inflammation of the blood vessel walls in the brain. The inflamed vessel wall
can block the flow blood and therefore oxygen to the brain, and cause a loss of brain function. It may produce a wide
range of neurological symptoms, such as headache, seizures, difficulty moving or coordinating part of the body,
changes in sensation, and alterations in perception, thought or behavior.
CAUSES
How the vessels in the brain become inflamed is not entirely clear. Usually, the disease occurs in the setting of other
medical conditions: infections, connective tissue diseases such as systemic lupus erythematosus (SLE) and rheumatoid
arthritis, particular medications and drugs (amphetamine, cocaine and heroin), and certain forms of cancer
(particularly lymphomas, leukemia and lung cancer). In different vasculitic diseases, our immune system forms
abnormal antibodies that eventually causes inflammation and destruction of the blood vessel wall. When no clear
underlying cause is found, the vasculitis is considered Primary CNS vasculitis, also called Primary Angiitis of the CNS
(PACNS).
Diagnosis
Cerebral vasculitis is a very rare condition that is difficult to diagnose, and as a result there are significant variations in
the way it is diagnosed and treated. Cerebral angiography (a procedure where contrast dye is injected in head and
neck blood vessels) and magnetic resonance imaging (non-invasive method of visualizing brain blood vessels), and
ultimately biopsy of the brain, are often required for its diagnosis. On cerebral angiography, CNS vasculitis is
characterized by “beading” (dilated areas alternating with narrowing of the blood vessels). A strikingly abnormal
angiogram may eliminate the need for a brain biopsy.
Treatment
CNS vasculitis is usually treated with medicines that quiet the immune system such as steroids. High-dose steroids
such as prednisone, in combination with cyclophosphamide (a medication that decreases the immune system’s
response to autoimmune diseases), are generally used. In some cases, high-dose steroids alone are tried first; if that
does not treat the disease, cyclophosphamide is added. Treatment must be continued for a prolonged period,
sometimes for life. If the patient has another illness (such as lupus) that is related to the vasculitis, then that illness
also needs to be treated.