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Images in Neurology |

Primary Cerebral Angiitis Associated With Amyloid Angiopathy

Amer A. Ghavanini, MD, PhD; David G. Munoz, MD, FRCPC
Arch Neurol. 2011;68(9):1202-1203. doi:10.1001/archneurol.2011.199.
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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.

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Figure 1. Magnetic resonance images. A, T2–fluid-attenuated inversion recovery (T2-FLAIR) image shows subcortical and cortical hyperintensity in the left frontal lobe. B, Gadolinium-enhanced T1 image shows abnormal enhancement of the leptomeninges covering the areas of T2-FLAIR abnormality (arrowheads). C, Gradient-echo images show multiple foci of microbleeding limited to the areas of T2-FLAIR hyperintensity and sparing the rest of the brain (arrows). Follow-up images 1 month after treatment with high-dose corticosteroids show markedly reduced T2-FLAIR hyperintensity (D), resolved leptomeningeal T1-gadolinium enhancement (E), and unchanged foci of microbleed gradient echo (F).

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Figure 2. Brain biopsy. A, Thickening of vessel walls with perivascular and mural infiltration of inflammatory cells and deposition of amorphous eosinophilic material of unknown nature (asterisks) (hematoxylin phloxine saffron). Bar indicates 100 μm. B, The perivascular and mural infiltrate consists of lymphocytes, eosinophils (arrows), and epithelioid macrophages. Deposition of amorphous eosinophilic material (asterisk) is seen (hematoxylin phloxine saffron). Bar indicates 25 μm. C, Hemosiderin-laden macrophages in the perivascular areas are indicative of microbleedings (arrowhead). Bar indicates 25 μm. D, Multinucleated giant-cells (double arrowheads) along with eosinophils (seen in B) are consistent with granulomatous inflammation. Bar indicates 25 μm. E, Martius-scarlet-blue staining shows fibrinoid necrosis of the vascular wall (arrow). Bar indicates 50 μm. F, Congo-red staining (polarized light) demonstrates amyloid deposits (arrows). Bar indicates 50 μm. G, Immunostaining with β-amyloid antibody shows vascular deposits (arrows), as well as plaques within the cortex (arrowhead). Bar indicates 50 μm. H, Higher-powered image shows co-localization of β-amyloid deposits (arrow) and vasculitis. Bar indicates 50 μm.




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