Teaching Neuro Images : Treatment-resistant rapidly progressive amyloid β–related angiitis

Abstract

A 76-year-old woman presented with 1 month of progressive aphasia, headache, and subsequent right hemiparesis. Initial brain MRI showed a punctate infarct (figure 1, A and C). Susceptibility-weighted imaging was unremarkable. A repeat study 16 days later demonstrated bihemispheric infarcts with multifocal attenuation of intracranial vessels on magnetic resonance angiography (figure 1, B and D). CSF showed a lymphocytic pleocytosis (101 leukocytes/µL) and elevated protein (480 mg/dL). Brain biopsy showed granulomatous angiitis with amyloid deposition and fibrinoid necrosis surrounded by inflammatory cells (figure 2). She rapidly deteriorated on immunosuppression with high-dose IV steroids and has not improved despite a combination of oral steroids and monthly cyclophosphamide.