Amyloid-β-related angiitis: a report of 2 cases with unusual presentations.
Amyloid-β-related angiitis (ABRA) is a rare complication of cerebral amyloid angiopathy in which amyloid-β deposition in the leptomeningeal and cortical vessels is associated with vasculitis characterized by transmural lymphohistiocytic, often granulomatous, inflammation. Patients usually present with acute to subacute cognitive dysfunction, headaches, and focal neurologic deficits. We report 2 cases of ABRA with unusual clinical presentations, including one case with fatal cerebral edema leading to herniation and Duret hemorrhages, and another associated with both lobar and deep parenchymal hemorrhages with intraventricular extension as well as hypercoagulability. Both showed extensive vascular amyloid-β deposition associated with granulomatous angiitis and foreign body-type multinucleated giant cells. One of our cases demonstrates the likely effects of ABRA on impairment of fluid regulation leading to severe cerebral edema, which is an uncommon manifestation of ABRA, and may be a result of impaired blood-brain barrier function or malfunction of the neurovascular unit.
Section of Neuropathology, Department of Pathology and Laboratory Medicine, University of California, Los Angeles (UCLA) Medical Center and David Geffen School of Medicine, Los Angeles, CA 90095-1732.
Denise W
DW
Ng
Section of Neuropathology, Department of Pathology and Laboratory Medicine, University of California, Los Angeles (UCLA) Medical Center and David Geffen School of Medicine, Los Angeles, CA 90095-1732. Electronic address: smagaki@mednet.ucla.edu.
Shino
S
Magaki
Section of Neuropathology, Department of Pathology and Laboratory Medicine, University of California, Los Angeles (UCLA) Medical Center and David Geffen School of Medicine, Los Angeles, CA 90095-1732.
Kevin H
KH
Terashima
Department of Neurology, University of California, Los Angeles (UCLA) Medical Center and David Geffen School of Medicine, Los Angeles, CA 90095-1732.
Adrienne M
AM
Keener
Department of Radiological Sciences, University of California, Los Angeles (UCLA) Medical Center and David Geffen School of Medicine, Los Angeles, CA 90095-1732.
Noriko
N
Salamon
Department of Radiological Sciences, University of California, Los Angeles (UCLA) Medical Center and David Geffen School of Medicine, Los Angeles, CA 90095-1732.
Stellios
S
Karnezis
Department of Neurosurgery, University of California, Los Angeles (UCLA) Medical Center and David Geffen School of Medicine, Los Angeles, CA 90095-1732.
Luke
L
Macyszyn
Section of Neuropathology, Department of Pathology and Laboratory Medicine, University of California, Los Angeles (UCLA) Medical Center and David Geffen School of Medicine, Los Angeles, CA 90095-1732; Department of Neurology, University of California, Los Angeles (UCLA) Medical Center and David Geffen School of Medicine, Los Angeles, CA 90095-1732; Brain Research Institute, University of California, Los Angeles (UCLA) Medical Center and David Geffen School of Medicine, Los Angeles, CA 90095-1732.
Harry V
HV
Vinters
D000368:Aged; D016229:Amyloid beta-Peptides / Q000032:analysis*; D001344:Autopsy; D001706:Biopsy; D001921:Brain / Q000378:metabolism* / Q000473:pathology; D001929:Brain Edema / Q000209:etiology; D016657:Cerebral Amyloid Angiopathy / Q000150:complications / Q000378:metabolism* / Q000473:pathology / Q000628:therapy; D002543:Cerebral Hemorrhage / Q000209:etiology; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D058225:Plaque, Amyloid
D016229:Amyloid beta-Peptides
Amyloid-β–related angiitis; Autopsy; Brain biopsy; Central nervous system; Cerebral amyloid angiopathy; Immunohistochemistry