Author Affiliations: Division of Neurology, Faculty of Medicine (Dr Ghavanini), and Division of Pathology, Department of Laboratory Medicine and Pathobiology (Dr Munoz), University of Toronto, Toronto, Ontario, Canada.
A 74-year-old woman had seizure and postictal right-sided weakness. She had a history of hypertension and a remote (>30 years) stroke with complete recovery. Family members reported cognitive changes during the preceding months including aggression, which was unusual for her calm personality. She was disoriented and refused to follow commands. Activities of daily living were severely impaired. Magnetic resonance imaging showed subcortical and cortical edema in the left frontal lobe on T2–fluid-attenuated inversion recovery images and gadolinium enhancement of the covering leptomeninges (Figure 1A and B). Gradient-echo magnetic resonance imaging showed foci of microbleedings limited to the area of edema, sparing the rest of the brain (Figure 1C). Extensive inflammatory investigations including erythrocyte sedimentation rate, antinuclear antibody, and antineutrophil cytoplasmic antibodies yielded negative findings. A biopsy specimen of the affected brain and adjacent leptomeninges (Figure 2) showed mural and perivascular infiltration by lymphocytes, eosinophils, epithelioid macrophages, and multinucleated giant cells associated with nuclear dust and fibrinoid necrosis consistent with primary cerebral angiitis. Congo-red staining demonstrated deposition of amyloid in the vessel walls. Immunostaining with β-amyloid antibody confirmed the β-amyloid nature of deposits in inflamed leptomeningeal and cortical vessels and the presence of numerous cortical amyloid plaques. She received intravenous corticosteroid therapy followed by a combination of oral prednisone and intermittent intravenous cyclophosphamide. Seizure and aggressive behavior were treated with phenytoin and quetiapine, respectively. At 1 month, she had marked radiological improvement (Figure 1D and E). At 6 months, she was calm, oriented, and able to follow commands, read, write, and perform simple activities of daily livings. Relapse following discontinuation of prednisone therapy at 6 months required its reinstatement.
Ghavanini AA, Munoz DG. Primary Cerebral Angiitis Associated With Amyloid Angiopathy. Arch Neurol. 2011;68(9):1202-1203. doi:10.1001/archneurol.2011.199