What is Cerebral Vasculitis?

Cerebral vasculitis is also commonly referred to as central nervous system (CNS) vasculitis. Vasculitis means that the blood vessels are inflamed. With this condition, the affected blood vessels are restricted to the spinal cord and brain. This condition is rare, but potentially life-threatening.

There are two broad categorizations of CNS vasculitis. It may be considered primary, also known as primary angiitis of the central nervous system (PACNS), or it may be secondary. Secondary CNS vasculitis may be associated with another disease, or it may be a reaction to drugs such as cocaine, amphetamines, or over-the-counter cold medicine. Cerebral vasculitis is classified as primary when no other existing diseases may be causing it. Primary CNS vasculitis is more rare than secondary.

The symptoms of this disorder often mimic other conditions, which may delay initial diagnosis. When the condition causes a blockage or reduction of blood flow to the brain, patients experience symptoms similar to a stroke. Symptoms may also mimic those of atherosclerosis, multiple sclerosis, or dislodged blood clots. These symptoms can cyclically heighten and diminish over a period of weeks or months.

Patients may experience periods of vision loss and speech impairment. They may also lose function in a leg or an arm. Patients often experience severe headaches that are not easily relieved. Some patients with this type of vasculitis may experience memory loss, confusion, and problems controlling bladder and bowel functions.

Cerebral vasculitis is often difficult to diagnose and may require a battery of tests. Doctors may use a computerized tomography (CT) scan and a magnetic resonance imaging (MRI) scan. A spinal tap, or analysis of cerebrospinal fluid, may also point to a possible neurological disease. Additional tests will be needed to confirm a diagnosis if CNS vasculitis is suspected.

Patients who may have this condition may undergo a brain biopsy, in which the surgeon takes a small sample of brain tissue to analyze. This procedure is performed while the patient is unconscious under general anesthesia. While a brain biopsy is a serious procedure, it may be life-saving if the patient is ill.

If the biopsy confirms CNS vasculitis, the patient will need to be treated with aggressive drug therapy. Examples of medications that are often used are glucocorticoids and cyclophosphamide. The patient is typically treated with these drugs for six to 12 months.

These medications do have the potential for serious side effects. Cyclophosphamide drugs can suppress the immune system, which renders a patient more vulnerable to infections and even cancer. Glucocorticoids may cause bone thinning, blood sugar and blood pressure abnormalities, and the risk of infections. Despite these side effects, the benefits often outweigh the risks because cerebral vasculitis is often deadly when it is left untreated.