CNS Vasculitis (Primary Angiitis of CNS)
Overview
CNS Vasculitis (aka primary angiitis of the central nervous system)
- Idiopathic
- Typically, recurrent
- Limited to the CNS & Dura
- Small/Medium-sized vasculopathy (vasculitis)
Typical Presentation
- Headache that is typically insidious/chronic
- Consider that thunderclap headache, instead, is typical for reversible cerebral vasoconstriction syndrome (RCVS)
- Mild encephalopathy or multifocal, variable symptomatology is common
- Occurs equally amongst males and females
- Typically presents in the 4th or 5th decade of life.
Diagnosis
- Definite diagnosis requires brain biopsy with demonstration of inflammation of small and/or medium-sized vessel walls within the brain parenchyma or leptomeninges.
- Abnormal cerebral angiography (characteristic "beading") and abnormal CSF testing (elevated WBC and elevated protein) can only give a high probability of the disorder; brain biopsy is necessary for a definitive diagnosis.
Treatment
- High-dose steroids +/- cyclophosphamide followed by chronic immune suppression (eg, azathioprine or mycophenolate mofetil).