Central nervous system (CNS) vasculitis is a rare disease that causes inflammation of the small arteries and veins in the brain and/or spinal cord. The brain and spinal cord make up the CNS.
The disease affects fewer than three individuals per one million, regardless of sex or age, and is associated with an overactive immune system that attacks the blood vessels in the brain. The inflamed vessel wall can block the flow of oxygen-containing blood to the brain, resulting in a loss of brain function, or stroke. CNS vasculitis can be caused by an underlying autoimmune disease (such as Sjogren’s syndrome or lupus), or it can develop independently.
Because CNS vasculitis is extremely rare, there is much to learn about why it occurs and how it presents.
Symptoms
Symptoms of CNS vasculitis may include:
- New, severe headaches
- Strokes or transient ischemic attacks (mini-strokes)
- Significant forgetfulness or confusion
- Weakness, especially of the limbs
- Loss of balance and gait disturbance
- Vision loss
- Seizures
- Sensation abnormalities (numbness and tingling, especially on one side of the body
Diagnosis
CNS vasculitis is often diagnosed through:
- Magnetic resonance imaging (MRI) scan of the brain with contrast
- Magnetic resonance (MR) or computed tomography (CT) angiography
- Conventional angiography
- Lumbar puncture (spinal tap)
- Brain biopsy
Treatment
Patients diagnosed with CNS vasculitis typically follow with a vascular (stroke) neurologist and a rheumatologist. Most are treated with steroids and other immunosuppressant drugs, which may be prescribed on a long-term basis.
Physical and occupational therapy can be important for patients with motor or gait deficits. Involvement of speech-language pathologists is recommended for patients with speech, language or cognitive issues.