Cerebral vasculitis is also commonly known as central nervous system (CNS) vasculitis . Vasculitis means that the blood vessels are inflamed. With cerebral vasculitis, the affected blood vessels are restricted to the spinal cord and brain. This condition is rare, but life-threatening.
There are two broad categorizations of CNS vasculitides. It can be considered primary, also known as primary central nervous system angiitis (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 medications. Cerebral vasculitis is classified as primary when no other existing disease can be causing it. Primary CNS vasculitis is rarer than secondary.
The symptoms of cerebral vasculitis often mimic other conditions, which can delay the initial diagnosis. When the condition causes a blockage or reduction of blood flow to the brain, patients experience symptoms similar to a stroke. Symptoms can also mimic those of atherosclerosis, multiple sclerosis, or dislodged blood clots. These symptoms may wax and wane cyclically over a period of weeks or months.
Patients may experience periods of vision loss and speech problems. They may also lose function in a leg or arm. Patients often experience severe headaches that are not easily relieved. Some patients with cerebral 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 computed tomography (CT) and magnetic resonance imaging (MRI). A lumbar puncture, or analysis of the cerebrospinal fluid, can also indicate possible neurological disease. Additional tests will be needed to confirm a diagnosis if CNS vasculitis is suspected.
Patients who may have cerebral vasculitis may undergo a brain biopsy, in which the surgeon removes a small sample of brain tissue for analysis. This procedure is performed while the patient is unconscious under general anesthesia. Although a brain biopsy is a serious procedure, it can be life-saving if the patient is ill.
If biopsy confirms CNS vasculitis, the patient should be treated with aggressive drug therapy. Examples of commonly used medications are glucocorticoids and cyclophosphamide. The patient is usually treated with these drugs for six to 12 months.
These medications have the potential to have serious side effects. Cyclophosphamide drugs can suppress the immune system, making the patient more vulnerable to infection and even cancer. Glucocorticoids can 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 fatal when left untreated.