Cerebral vasculitis

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Cerebral vasculitis
Classification and external resources
ICD-10 I67.7
ICD-9 437.4
MeSH D020293

Cerebral vasculitis or central nervous system vasculitis (sometimes the word angiitis is used instead of "vasculitis") is vasculitis (inflammation of the blood vessel wall) involving the brain and occasionally the spinal cord. It affects all of the vessels: very small blood vessels (capillaries), medium-size blood vessels (arterioles and venules), or large blood vessels (arteries and veins). If blood flow in a vessel with vasculitis is reduced or stopped, the parts of the body that receive blood from that vessel begins to die. It may produce a wide range of neurological symptoms, such as headache, skin rashes, feeling very tired, joint pains, difficulty moving or coordinating part of the body, changes in sensation, and alterations in perception, thought or behavior, as well as the phenomena of a mass lesion in the brain leading to coma and herniation. Some of its signs and symptoms may resemble multiple sclerosis.[1] 10% have associated bleeding in the brain.[2]


"Primary" Central Nervous System (CNS) vasculitis is said to be present if there is no underlying cause. However, the immune system is known to play a part in the cause of the disease. This is because sometimes the immune system becomes over reactive and attacks the host's body. There are often antigens (medicines or illness that cause an allergic reaction) that are factors that help lead to this disease. Some of these possible antigens are infections, other forms of systemic vasculitis such as granulomatosis with polyangiitis or polyarteritis nodosa, connective tissue diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis, particular medications and drugs (amphetamine, cocaine and heroin), certain forms of cancer (particularly lymphomas, leukemia and lung cancer) and Behçet's disease. It may imitate, and is in turn imitated by, a number of other diseases that affect the blood vessels of the brain diffusely such as fibromuscular dysplasia and thrombotic thrombocytopenic purpura.[2]


Cerebral angiography and magnetic resonance imaging, family medical history, symptoms, a complete physical examination, and ultimately biopsy of the brain, are often required for the diagnosis. Also, many lab tests must be done for the diagnosis: tests may reveal anemia (a shortage of red blood cells), a high white blood cell count, a high platelet count, allergic reactions, immune complexes, antibodies (tools the body uses to fight off threats), and elevation of inflammatory markers. Another crucial part in the diagnosis of cerebral vasculitis is the use of imaging techniques. Techniques such as conventional digital subtraction angiography (DSA) and magnetic resonance imaging (MRI) are used to find and monitor cerebral involvement.


Treatment is first with many different high-dose steroids from prednisone to glucocorticoids. Then often if symptoms do not improve additional medications such as immunosuppression for example cyclophosphamide are added to decrease the immune system's attack on the body's own tissues.[2] 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.[3]


  1. ^ Scolding NJ, Jayne DR, Zajicek JP, Meyer PA, Wraight EP, Lockwood CM (January 1997). "Cerebral vasculitis--recognition, diagnosis and management". QJM 90 (1): 61–73. doi:10.1093/qjmed/90.1.61. PMID 9093590. 
  2. ^ a b c Rehman HU (November 2000). "Primary angiitis of the central nervous system" (PDF). J R Soc Med 93 (11): 586–8. PMC 1298150. PMID 11198690. 
  3. ^ Scolding NJ, Wilson H, Hohlfeld R, Polman C, Leite I, Gilhus N (July 2002). "The recognition, diagnosis and management of cerebral vasculitis: a European survey". Eur. J. Neurol. 9 (4): 343–7. doi:10.1046/j.1468-1331.2002.00422.x. PMID 12099915. 

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