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Primary CNS angiitis (PCNSA) is a focal or multifocal vasculitis with predilection for small arteries that is restricted to the brain or spinal cord.1,–,3 Both diffuse lymphocytic and granulomatous inflammatory patterns are noted, which may or may not go along with focal necrosis.2,4,5 About 30% of patients with PCNSA exhibit prominent histopathologic signs of cerebral amyloid angiopathy (CAA).2,4 Compared with other patients, patients with β-amyloid (Aβ)-related angiitis (ABRA) are older and have a more acute disease onset with a high prevalence of gadolinium-enhanced MRI lesions,4 suggesting that CAA predisposes to an exacerbated inflammatory response. Whether ABRA shares the same pathomechanisms as other forms of PCNSA or belong to a different disease entity was unknown.
Case report.
A 67-year-old man presented with psychomotor slowing, memory problems, aphasia, right-sided limb …
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