An article in the Lancet (2012, 380: 767–77) provides an overview on the current state of diagnosis and treatment of primary CNS vasculitis. It was written by Carlo Salvarani, Department of Internal Medicine, Azienda Ospedaliera ASMN, Istituto di Ricoveroe Cura a Carattere Scientifico, Reggio Emilia, Italy; Robert Brown Jr., Department of Neurology, Mayo Clinic, Rochester, MN, USA; and GeneHunder, College of Medicine, Mayo Clinic, Rochester, MN, USA.
First chronicled in the 1950s, doctors still don’t know what exactly causes primary CNS vasculitis. This uncommon disorder affects only the brain and spinal cord. The median age of onset is 50, although it can occur in children.
CNSV is hard to diagnose because its many symptoms also occur in more common conditions. It often presents with headache, trouble focusing, altered cognition and even stroke. Brain MRIs are usually normal. For this reason, there are three suggested criteria for a confirmed diagnosis:
1. History of or clinical findings of an acquired neurological deficit of unknown origin after a thorough initial basic assessment.
2. Cerebral angiogram with classic features of vasculitis or a CNS biopsy sample showing vasculitis. (Cerebral and meningeal biopsy is considered the “gold standard” diagnostic test.)
3. No evidence of systemic vasculitis or other disorder with similar pathological or angiographic features.
Getting a correct and early diagnosis is important because the condition can be effectively treated – and further complications avoided – with corticosteroids, sometimes paired with cytotoxic drugs. There is growing evidence that patients with primary CNSV respond differently to treatments, but no randomized therapeutic clinical trials have been done to confirm what works best. Currently, treatments vary depending on the size of the vessels involved and are based on:
• Therapies used to treat other forms of vasculitis.
• Anecdotal reports.
• Cohort studies.
Creating an international collaborative registry for primary CNSV could lead to standardized classifications and diagnostic criteria, and help clinicians differentiate the disease from conditions with similar presentations. It also could aid in defining patient groups for randomized clinical trials, and build a biological specimen bank to support additional translational research. These activities would help the medical community develop a better understanding of the disease and its treatment.