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An autopsy case of amyloid β-related angiitis with cognitive impairment, multiple infarcts and subcortical hemorrhage

Published on Nov 1, 2018in Human Pathology: Case Reports
· DOI :10.1016/j.ehpc.2018.07.007
Takahiro Watanabe6
Estimated H-index: 6
(Hyogo College of Medicine),
Shinichiro Ukon (Hyogo College of Medicine)+ 12 AuthorsYoshitane Tsukamoto24
Estimated H-index: 24
(Hyogo College of Medicine)
Abstract A 76-year-old female presented with cognitive impairment and multiple brain infarcts and died of massive subcortical cerebral hemorrhage. A premortem clinical diagnosis was not possible. She did not show apparent microbleeds on brain images during her lifetime. An autopsy revealed vasculitis at various stages with amyloid β deposition and granulomatous reaction in cortical and leptomeningeal arteries, although almost vasculitic processes were in the healing stage, suggestive of the inactive stage of the disease process. Affected vessels were strictly limited to the brain. The final pathological diagnosis of the autopsy was amyloid β-related angiitis. Genotype analyses of ApoE revealed ApoE e3/e3 genotype, low risk for Alzheimer's disease. On retrospective examination, the titer of anti-amyloid β autoantibody in cerebrospinal fluid was rather high in our case, but the exact clinical significance remains unknown. Since microbleeds on brain images are diagnostic findings in patients with cerebral amyloid angiopathy-associated conditions, it is very difficult to make an accurate diagnosis of amyloid β-related angiitis without typical microbleeds or open brain biopsy like our case.
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Published on Jun 1, 2017in Human Pathology 2.74
Denise W. Ng3
Estimated H-index: 3
(UCLA: University of California, Los Angeles),
Shino Magaki9
Estimated H-index: 9
(UCLA: University of California, Los Angeles)
+ 5 AuthorsHarry V. Vinters94
Estimated H-index: 94
(UCLA: University of California, Los Angeles)
Summary 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 e...
Published on Feb 1, 2017in Journal of Neurology 4.20
Akio Kimura4
Estimated H-index: 4
(Gidai: Gifu University),
Masao Takemura5
Estimated H-index: 5
(Gidai: Gifu University)
+ 6 AuthorsTakashi Inuzuka22
Estimated H-index: 22
(Gidai: Gifu University)
Brain magnetic resonance imaging (MRI) of patients with Alzheimer’s disease (AD) sometimes reveals multiple cerebral microbleeds (CMBs) and confluent white matter hyperintensities (WMHs) similar to those observed in cerebral amyloid angiopathy-related inflammation (CAA-I). To determine whether there might be common pathophysiological mechanisms underlying the MRI findings of multiple CMBs and confluent WMHs, we investigated the cerebrospinal fluid (CSF) profiles of 38 AD, five amnestic mild cogn...
Published on Dec 1, 2016in Brain 11.81
Susanne J. van Veluw17
Estimated H-index: 17
(Harvard University),
Andreas Charidimou30
Estimated H-index: 30
(Harvard University)
+ 8 AuthorsAnand Viswanathan47
Estimated H-index: 47
(Harvard University)
Cerebral amyloid angiopathy is a common neuropathological finding in the ageing human brain, associated with cognitive impairment. Neuroimaging markers of severe cerebral amyloid angiopathy are cortical microbleeds and microinfarcts. These parenchymal brain lesions are considered key contributors to cognitive impairment. Therefore, they are important targets for therapeutic strategies and may serve as surrogate neuroimaging markers in clinical trials. We aimed to gain more insight into the patho...
Published on Aug 1, 2016in Acta Neuropathologica 18.17
Mitsuru Shinohara17
Estimated H-index: 17
(Mayo Clinic),
Melissa E. Murray44
Estimated H-index: 44
(Mayo Clinic)
+ 15 AuthorsChia-Chen Liu22
Estimated H-index: 22
(Mayo Clinic)
Cerebral amyloid angiopathy (CAA) often coexists with Alzheimer’s disease (AD). APOE4 is a strong genetic risk factor for both AD and CAA. Sex-dependent differences have been shown in AD as well as in cerebrovascular diseases. Therefore, we examined the effects of APOE4, sex, and pathological components on CAA in AD subjects. A total of 428 autopsied brain samples from pathologically confirmed AD cases were analyzed. CAA severity was histologically scored in inferior parietal, middle frontal, mo...
Published on May 1, 2016in Medicine 1.87
Carlo Salvarani69
Estimated H-index: 69
Jonathan M. Morris22
Estimated H-index: 22
+ 3 AuthorsGene G. Hunder76
Estimated H-index: 76
(Mayo Clinic)
Vascular inflammation is present in a subset of patients with cerebral amyloid angiopathy (CAA) and has a major influence in determining the disease manifestations. Radiological characterization of this subset is particularly important to achieve early recognition and treatment. We conducted this study to investigate the role of imaging in differentiating CAA with and without inflammation. We reviewed neuroimaging findings for 54 patients seen at Mayo Clinic over 25 years with pathological evide...
Published on Sep 1, 2015in Genetics in Medicine 8.68
Sumit Parikh15
Estimated H-index: 15
(Cleveland Clinic),
Amy Goldstein16
Estimated H-index: 16
(University of Pittsburgh)
+ 16 AuthorsCarol L. Greene24
Estimated H-index: 24
(UMB: University of Maryland, Baltimore)
Diagnosis and management of mitochondrial disease: a consensus statement from the Mitochondrial Medicine Society
Published on Jan 1, 2015in Journal of stroke 5.57
Masahito Yamada1
Estimated H-index: 1
(Kanazawa University)
Cerebral amyloid angiopathy (CAA) involves cerebrovascular amyloid deposition and is classified into several types according to the amyloid protein involved. Of these, sporadic amyloid β-protein (Aβ)-type CAA is most commonly found in older individuals and in patients with Alzheimer's disease (AD). Cerebrovascular Aβ deposits accompany functional and pathological changes in cerebral blood vessels (CAA-associated vasculopathies). CAA-associated vasculopathies lead to development of hemorrhagic le...
Published on Aug 1, 2014in Seminars in Arthritis and Rheumatism 5.07
Abhijeet Danve7
Estimated H-index: 7
(OHSU: Oregon Health & Science University),
Marjorie R. Grafe25
Estimated H-index: 25
(OHSU: Oregon Health & Science University),
Atulya (Atul) Deodhar18
Estimated H-index: 18
(OHSU: Oregon Health & Science University)
Abstract Background Amyloid Beta-Related Angiitis (ABRA) is a rare cause of central nervous system vasculitis complicating cerebral amyloid angiopathy. Data regarding its prevalence, clinical features, management, and outcomes are scant. Objectives To describe a patient with ABRA and discuss clinical features and management of ABRA. Methods A case report and review of literature were conducted of all reported cases of ABRA in the English literature. Results The exact etiology of ABRA is not clea...
Published on Jan 1, 2014in Frontiers in Neurology 2.63
Amre Nouh3
Estimated H-index: 3
(Loyola University Medical Center),
Ewa Borys5
Estimated H-index: 5
(Loyola University Medical Center)
+ 1 AuthorsJosé Biller53
Estimated H-index: 53
(Loyola University Medical Center)
Amyloid-beta related angiitis (ABRA) of the central nervous system (CNS) is a rare disorder with overlapping features of primary angiitis of the CNS and cerebral amyloid angiopathy. We evaluated a 74-year-old man with intermittent left sided weakness and MRI findings of leptomeningeal enhancement, vasogenic edema, and subcortical white matter disease proven to have ABRA. We discuss clinicopathological features and review the topic of ABRA.
Published on Dec 1, 2013in Journal of Neuroinflammation 5.70
Akio Kimura16
Estimated H-index: 16
(Gidai: Gifu University),
Takeo Sakurai12
Estimated H-index: 12
(Gidai: Gifu University)
+ 4 AuthorsTakashi Inuzuka22
Estimated H-index: 22
(Gidai: Gifu University)
We studied longitudinal changes of the levels of anti-amyloid β (anti-Aβ) antibody, amyloid β (Aβ) protein, and interleukin 8 (IL-8) in cerebrospinal fluid (CSF) of a patient with cerebral amyloid angiopathy-related inflammation (CAA-ri) in whom steroid treatment resulted in clinical improvement. The diagnosis of CAA-ri was established with brain biopsy. Levels of anti-Aβ 42 antibody, Aβ 40, Aβ 42 and IL-8 in CSF were measured in the CAA-ri patient at 23 time points in the 8-month clinical cours...
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