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Central Nervous System (CNS) Vasculitis

What is central nervous system vasculitis?

Central nervous system (CNS) vasculitis refers to vasculitis in the brain. The condition can be primary, with manifestations of vasculitis limited to the brain, or secondary, with brain involvement with a systemic form of vasculitis such as polyarteritis nodosa (PAN), granulomatosis with polyangiitis (Wegener’s), or others. The most typical symptoms of either primary or secondary CNS vasculitis include headache and cognitive dysfunction but may also include any of the following: seizure, stroke and coma.

Who gets central nervous system vasculitis?

Primary CNS vasculitis is extremely rare (less than 1-2 persons per million people per year. Men and women of all ages can get the disease although it is slightly more frequent in middle-aged men (45-65). Five to 15 percent of patients with PAN and granulomatosis with polyangiitis (Wegener’s) will get central nervous system manifestations (secondary vasculitis).

What causes central nervous system vasculitis?

The cause of primary CNS vasculitis is unknown as are the causes of the diseases associated with secondary CNS vasculitis.

How is central nervous system vasculitis diagnosed?

Since primary CNS vasculitis is so rare, a high index of suspicion is required to make the diagnosis. Mimickers must be ruled out, including drug use (ephedrine, cocaine, etc.), migraine, infections and some unusual malignancies. A combination of tests, including magnetic resonance imaging (MRI), cerebral angiography and spinal fluid examination (spinal tap) may help. In many cases, a brain biopsy is the only way to confirm the diagnosis.

What is the treatment for central nervous system vasculitis?

Depending on the clinical manifestations and extent of the disease, patients with CNS vasculitis will usually require treatment with corticosteroids combined with immunosuppressive agents, such as cyclophosphamide or azathioprine, for a period varying between six and 12 months.