Cerebral vasculitis
Other namesCentral nervous system vasculitis
SpecialtyCardiology, neurology, rheumatology Edit this on Wikidata

Cerebral 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.[1] 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.[2] 10% have associated bleeding in the brain.[3]

Causes

"Primary" angiitis/vasculitis of the central nervous system (PACNS) is said to be present if there is no underlying cause. The exact mechanism of the primary disease is unknown, but the fundamental mechanism of all vasculitides is autoimmunity. Other possible secondary causes of cerebral vasculitis are infections, systemic auto-immune diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis, medications and drugs (amphetamine, cocaine and heroin), some forms of cancer (lymphomas, leukemia and lung cancer) and other forms of systemic vasculitis such as granulomatosis with polyangiitis, polyarteritis nodosa or 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.[3]

Diagnosis

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.[4] 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.[citation needed]

Treatment

Treatment is first with many different high-dose steroids, namely glucocorticoids. Then, if symptoms do not improve additional immunosuppression such as cyclophosphamide are added to decrease the immune system's attack on the body's own tissues.[3] 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.[5]

Specific Diseases

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Giant cell arteritis (GCA)

(Also known as temporal arteritis)

Symptoms

Diagnostic criteria

Three or more of the following five criteria must be met:

Takayasu's arteritis

Symptoms

Starts with nonspecific symptoms such as:

Classification criteria

Three or more of the following six criteria must be met:

Treatment therapy

Polyarteritis nodosa (PAN)

Symptoms

Diagnostic Criteria

Three or more of the following ten criteria are required:

Treatment therapy

Granulomatosis with polyangiitis (GPA)

Symptoms

Diagnostic Criteria

Two or more of the following four criteria are required:

Treatment Therapy

References

  1. ^ "Cerebral Vasculitis". Prime Health Channel. 19 December 2012. Retrieved 1 May 2015.
  2. ^ 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.
  3. ^ a b c Rehman HU (November 2000). "Primary angiitis of the central nervous system". J R Soc Med. 93 (11): 586–8. doi:10.1177/014107680009301108. PMC 1298150. PMID 11198690. Archived from the original (PDF) on 2009-11-05. Retrieved 2008-07-23.
  4. ^ "Central Nervous System Vasculitis (CNS Vasculitis)". Cleveland Clinic. Retrieved 1 May 2015.
  5. ^ 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. S2CID 18859529.
  6. ^ Berlit, Peter (1 October 2010). "Diagnosis and treatment of cerebral vasculitis". Therapeutic Advances in Neurological Disorders. 3 (1): 29–42. doi:10.1177/1756285609347123. ISSN 1756-2864. PMC 3002614. PMID 21180634.

External links

Classification
Cerebrovascular diseases including stroke
Categories