University of Virginia School of Medicine



Primary Central Nervous System VasculitisEducation Content: central nervous system vasculitisCentral 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.?CAUSESHow 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.TreatmentCNS 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. ................
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