A 60-year-old woman presented with 3 weeks of headache and acute confusion, which resolved after intravenous hydration. She returned after a fall, drowsy, confused, weak on the left and aphasic. Work-up revealed elevated Sedimentation rate (34) and CRP (76). CSF had elevated protein (222), RBC (313), and corrected WBC (33). Vasculitis panel, serum and protein electrophoresis were unremarkable.
She received broad spectrum antibiotics and steroids. MRI Brain demonstrated scattered foci of restricted diffusion in bilateral hemispheres in different vasculatures, left parietal vasogenic edema, and punctate areas of enhancement concerning for atypical PRES, thrombotic microangiopathy or inflammatory amyloid angiopathy.
Biopsy showed reactive gliosis, perivascular inflammation with multinucleated giant cells and fibrinoid necrosis of blood vessels. Congo red staining highlighted amyloid. ABRA was confirmed and prescribed high dose Prednisone with 10-week taper; patient recovered significantly but lost to follow up.
A year later, she presented with seizure, hypothermia to 85 F and hypotension. MRI Brain showed multifocal bilateral punctate infarcts, confluent vasogenic edema in frontal, temporal, and left parieto-occipital lobes, with extensive microbleeds along subcortical-cortical margins. Arteriogram was unremarkable. Treatment with antibiotics, and daily intravenous Methylprednisone was started. By day 3, the patient was off vasopressor, transitioned to oral prednisone and started cyclophosphamide. She returned to baseline mentation and strength on 2 month follow up.