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Vasculitis, central nervous system

Vasculitis of the central nervous system (CNS) is an autoimmune disorder in which the body mistakes its own blood vessels that supply the brain and spinal cord as a foreign substance and launches antibodies to attack them. It is not known what causes this faulty immune response. Inflammation of the blood vessels prevents blood from flowing to parts of the brain and spinal cord, which can result in damage to the CNS, including:

  • decreased function due to decreased blood flow (ischemia),
  • death of some or all of an organ due to absent blood flow (infarction), or
  • bleeding into the skin or other part of the body due to rupture of the blood vessel wall.

Early detection and treatment can greatly help the vasculitis patient.

Symptoms

The inflammation of vasculitis is usually accompanied by other features such as fever, or symptoms of involuntary weight loss and fatigue.

Severe headaches, stepwise stroke-like focal symptoms, changes in mental status, difficulty with coordination can be indicative of brain vasculitis, while shooting pains in the arms and legs, numbness, and asymmetrical weakness (i.e., weakness that involves one side of the body more than the other) could point to vasculitis of the nervous system.

Diagnosis

Vasculitis of the central nervous system may be diagnosed using the following approaches:

  • Patients with evidence of nerve or muscle involvement may undergo nerve conduction studies and an electromyogram (a test of muscle function).
  • In vasculitis affecting the larger vessels, an arteriogram may be useful. This test involves injecting dye into the arteries, which makes them visible on x-ray.
  • A definitive diagnosis of CNS vasculitis is only possible with the aid of a brain biopsy. However, doctors can confirm their suspicion with
    • blood tests
    • examination of the cerebrospinal fluid through a spinal tap (lumbar puncture)
    • magnetic resonance imaging (MRI), and
    • cerebral angiography

Treatment

Fortunately, available treatments for vasculitis are helpful, especially in the acute phase. Long-term therapy presents challenges because of side effects of the medications typically used to treat the disorder.

Often, this disease responds to high-dose steroids. In some cases, chemotherapeutic agents which suppress the immune system are necessary.

Treatment also may require the removal and reinfusion of blood plasma (plasmapheresis) or the use of gammaglobulin.

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