Primary central nervous system vasculitis: clinical experiences with 21 new European cases

Primary central nervous system vasculitis (PCNSV) represents a rare and poorly understood form of vascular inflammatory disease restricted to the brain and spinal cord. This study was undertaken to better characterize demographic and clinical features and to evaluate treatments and outcome. Our cohort included 21 white patients with PCNSV treated in a single German institution between 2003 and 2008. Clinical and diagnostic features were obtained by retrospective chart review; follow-up information and outcome were obtained prospectively. Clinical findings and outcomes were compared among patients categorized by different characteristics. The mean age at onset of symptoms was 42.48 years. In 6 (28.6%) of 21 patients, PCNSV diagnosis was established based on positive CNS tissue histopathology. Additional angiography was only performed in two of these six patients and was negative in both cases. For 13 patients (61.9%), the diagnosis was confirmed by positive cerebral angiogram. The most frequent clinical presentations over the whole follow-up period were hemiparesis (76.2%), cerebral ischemia (66.7%), headache (57.1%) and altered cognition (52.4%). Patients with MR-tomographic evident microangiopathy showed significantly worse modified Rankin disability scores during the onset and at last visit compared to the other patients (P = 0.047, P = 0.039). Clinical features and course of PCNSV of Europeans analyzed in this German study are comparable to American results but some further reported characteristics of different subtypes could not be duplicated. Although this well-defined analysis adds to our knowledge of PCNSV, it also raises important open questions. The enigma of PCNSV could only be solved by implementing large prospective multicenter databases and elucidating animal models.