Aβ-related angiitis: Comparison with CAA without inflammation and primary CNS vasculitis

Objective: To analyze the clinical findings, response to therapy, and outcomes of patients with cerebral vascular amyloid-b (Ab) deposition with and without inflammatory vascular infiltration. Methods: We report 78 consecutive patients with cerebral vascular Ab deposition examined at Mayo Clinic Rochester over 25 years (1987 through 2011). Specimens reviewed by a neuropathologist showed 40 with vascular Ab peptide without inflammation (cerebral amyloid angiopathy [CAA]), 28 with granulomatous vasculitis (Ab-related angiitis or ABRA), and 10 with perivascular CAA-related inflammation. We also matched findings in 118 consecutive patients with primary CNS vasculitis (PCNSV) without Ab seen over 25 years (1983 through 2007). Results: Compared to the 40 with CAA, the 28 with ABRA were younger at diagnosis (p = 0.05), had less altered cognition (p = 0.02), fewer neurologic deficits (p = 0.02), and fewer intracranial hemorrhages (<0.001), but increased gadolinium leptomeningeal enhancement (p = 0.01) at presentation, and less mortality and disability at last follow-up (p < 0.001). Compared with PCNSV, the 28 patients with ABRAwere older at diagnosis (p < 0.001), had a higher frequency of altered cognition (p = 0.05), seizures/spells (p = 0.006), gadolinium leptomeningeal enhancement (p < 0.001), and intracerebral hemorrhage (p = 0.02), lower frequency of hemiparesis (p = 0.01), visual symptoms (p = 0.04), and MRI evidence of cerebral infarction (p = 0.003), but higher CSF protein levels (p = 0.03). Results of treatment and outcomes in ABRA and PCNSV were similar. Conclusions: ABRA appears to represent a distinct subset of PCNSV.