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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 may produce a wide range of neurological symptoms, such as headache, difficulty moving or coordinating part of the body, changes in sensation, and alterations in perception, thought or behaviour, as well as the phenomena of a mass lesion in the brain leading to coma and herniation. Some of its symptoms may resemble multiple sclerosis.[1] 10% have associated cerebral hemorrhage.[2]

Contents

Causes

"Primary" CNS vasculitis is said to be present if there is no underlying cause. More commonly, the disease occurs in the setting of other medical conditions: infections, other forms of systemic vasculitis such as Wegener's granulomatosis 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]

Diagnosis and treatment

Cerebral angiography and magnetic resonance imaging, and ultimately biopsy of the brain, are often required for its diagnosis. Once confirmed, various tests are often performed to establish whether the vasculitis is primary or the result of an underlying cause. Treatment is with glucocorticoids, often with additional immunosuppression with medication such as cyclophosphamide 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]

References

  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. 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. PMID 11198690. PMC 1298150. http://jrsm.rsmjournals.com/cgi/reprint/93/11/586.  
  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. http://www3.interscience.wiley.com/journal/118961115/abstract.  

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