Primary CNS Vasculitis: Often in the Differential but Rarely Confirmed

Onset of focal neurological symptoms, headaches, and seizures generates a broad differential diagnosis, and primary central nervous system (CNS) vasculitis often is invoked. In this cohort study from 21 hospitals in France, investigators present data on 52 patients in whom primary CNS vasculitis was diagnosed between 1996 and 2012 and for whom other potential diagnoses were excluded. Diagnoses were confirmed by brain biopsy or neuroimaging studies using either conventional cerebral angiography or magnetic resonance angiography.

At the time of diagnosis, about half of patients had new-onset headaches that preceded by a few days or months — or were associated with — other neurological symptoms (e.g., focal deficits, speech disorders, seizures)…