Autopsy of a multiple lobar hemorrhage case with amyloid-β-related angiitis.
amyloid β-related angiitis
amyloid-β protein
cerebral amyloid angiopathy
intracerebral hemorrhage
primary central nerve system vasculitis
Journal
Neuropathology : official journal of the Japanese Society of Neuropathology
ISSN: 1440-1789
Titre abrégé: Neuropathology
Pays: Australia
ID NLM: 9606526
Informations de publication
Date de publication:
Jun 2020
Jun 2020
Historique:
received:
21
10
2019
revised:
18
11
2019
accepted:
18
11
2019
pubmed:
22
1
2020
medline:
2
4
2021
entrez:
22
1
2020
Statut:
ppublish
Résumé
A 92-year-old man died of multiple lobar hemorrhage with amyloid-β protein (Aβ)-related angiitis (ABRA) with an unusual pathological appearance. Although he had shown relatively rapid progressive dementia, starting 1 year before death, there was no detailed clinical investigation, and no immunosuppressive or anticoagulant therapy, because of his advanced age. The autopsy showed two lobar hemorrhagic lesions in the right parietal lobe and temporal lobes. Microscopically, almost all the brain's blood vessels showed cerebral amyloid angiopathy with many foci of transmural vasculitis. Infiltrating cells were predominantly CD8-positive T-lymphocytes, but we observed no granulomatous inflammation with appearance of multinucleated giant cells. We found fibrinoid necrosis in some blood vessels and disruption of these blood vessels in the arachnoid space-cerebral cortex junction in the hemorrhagic lesion at the temporal lobe. We also observed an unusual, neutrophil-predominant, abscess-like vasculitis in the subarachnoid space; almost all such unusual vasculitides were located at a short distance from the two lobar hemorrhagic lesions. Serum anti-neutrophil cytoplasmic myeloperoxidase and proteinase-3 antibodies were negative, and the genotype of the apolipoprotein E (ApoE) gene (ApoE) was ε2/ε3. Although we did not observe some of ABRA's typical histopathological findings, transmural and vascular destructive inflammation with Aβ deposition was consistent with ABRA. Vulnerability of blood vessels to fibrinoid necrosis might be associated with disruption of the relevant blood vessels, leading to lobar hemorrhage. ABRA exhibits various clinical and histopathological findings, depending on the patient's age, immune function status, treatment, and ApoE genotype. This is the first case and the oldest (92 years old) autopsy of ABRA associated with ApoE-ε2/ε3 genotype.
Substances chimiques
Amyloid beta-Peptides
0
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
280-286Subventions
Organisme : Japan Society for the Promotion of Science
ID : JP17k09263
Organisme : Japan Society for the Promotion of Science
ID : JP18k10119
Informations de copyright
© 2020 Japanese Society of Neuropathology.
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