An autopsy case of amyloid β-related angiitis with cognitive impairment, multiple infarcts and subcortical hemorrhage
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 vari...
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| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2018-11-01
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| Series: | Human Pathology: Case Reports |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2214330018300865 |
| _version_ | 1818552682755915776 |
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| author | Takahiro Watanabe, MD, PhD Shinichiro Ukon, MD Takako Kihara, MD Yoshi-Hiro Ide, MD, PhD Shohei Matsuo, MD Yuka Hayashi, MD Shuhei Kasama, MD, PhD Hiroo Yoshikawa, MD, PhD Yuki Okano Chiaki Uchida Masahiro Yamamoto Hiroyuki Hao, MD, PhD Akio Kimura, MD, PhD Seiichi Hirota, MD, PhD Yoshitane Tsukamoto, MD, PhD |
| author_facet | Takahiro Watanabe, MD, PhD Shinichiro Ukon, MD Takako Kihara, MD Yoshi-Hiro Ide, MD, PhD Shohei Matsuo, MD Yuka Hayashi, MD Shuhei Kasama, MD, PhD Hiroo Yoshikawa, MD, PhD Yuki Okano Chiaki Uchida Masahiro Yamamoto Hiroyuki Hao, MD, PhD Akio Kimura, MD, PhD Seiichi Hirota, MD, PhD Yoshitane Tsukamoto, MD, PhD |
| author_sort | Takahiro Watanabe, MD, PhD |
| collection | DOAJ |
| description | 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 ε3/ε3 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. Keywords: Amyloid β-related angiitis, Cerebral amyloid angiopathy, ApoE, Subcortical cerebral hemorrhage, Primary central nervous system vasculitis |
| first_indexed | 2024-12-12T09:16:22Z |
| format | Article |
| id | doaj.art-5e53844ae67941b2b88668c95e126cda |
| institution | Directory Open Access Journal |
| issn | 2214-3300 |
| language | English |
| last_indexed | 2024-12-12T09:16:22Z |
| publishDate | 2018-11-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Human Pathology: Case Reports |
| spelling | doaj.art-5e53844ae67941b2b88668c95e126cda2022-12-22T00:29:23ZengElsevierHuman Pathology: Case Reports2214-33002018-11-01144751An autopsy case of amyloid β-related angiitis with cognitive impairment, multiple infarcts and subcortical hemorrhageTakahiro Watanabe, MD, PhD0Shinichiro Ukon, MD1Takako Kihara, MD2Yoshi-Hiro Ide, MD, PhD3Shohei Matsuo, MD4Yuka Hayashi, MD5Shuhei Kasama, MD, PhD6Hiroo Yoshikawa, MD, PhD7Yuki Okano8Chiaki Uchida9Masahiro Yamamoto10Hiroyuki Hao, MD, PhD11Akio Kimura, MD, PhD12Seiichi Hirota, MD, PhD13Yoshitane Tsukamoto, MD, PhD14Department of Surgical Pathology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, JapanDivision of Neurology, Department of Internal Medicine, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, JapanDepartment of Surgical Pathology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, JapanDepartment of Surgical Pathology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, JapanDepartment of Pathology and Laboratory Medicine, Takarazuka City Hospital, 4-5-1 Kohama, Takarazuka, Hyogo 665-0827, JapanDepartment of Neurology, Fukuoka University Hospital, 8-19-1 Nanakuma, Jonan-ku, Fukuoka 814-0180, JapanDivision of Neurology, Department of Internal Medicine, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, JapanDivision of Neurology, Department of Internal Medicine, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, JapanDepartment of Surgical Pathology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, JapanDepartment of Surgical Pathology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, JapanDepartment of Surgical Pathology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, JapanDepartment of Human Pathology, Nihon University School of Medicine, 30-1 Ooyaguchikami-cho, Itabashi-ku, Tokyo 173-8610, JapanDepartment of Neurology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, JapanDepartment of Surgical Pathology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, JapanDepartment of Surgical Pathology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, Japan; Department of Pathology and Laboratory Medicine, Takarazuka City Hospital, 4-5-1 Kohama, Takarazuka, Hyogo 665-0827, Japan; Corresponding author at: Department of Pathology and Laboratory Medicine, Takarazuka City Hospital, 4-5-1 Kohama, Takarazuka, Hyogo 665-0827, Japan.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 ε3/ε3 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. Keywords: Amyloid β-related angiitis, Cerebral amyloid angiopathy, ApoE, Subcortical cerebral hemorrhage, Primary central nervous system vasculitishttp://www.sciencedirect.com/science/article/pii/S2214330018300865 |
| spellingShingle | Takahiro Watanabe, MD, PhD Shinichiro Ukon, MD Takako Kihara, MD Yoshi-Hiro Ide, MD, PhD Shohei Matsuo, MD Yuka Hayashi, MD Shuhei Kasama, MD, PhD Hiroo Yoshikawa, MD, PhD Yuki Okano Chiaki Uchida Masahiro Yamamoto Hiroyuki Hao, MD, PhD Akio Kimura, MD, PhD Seiichi Hirota, MD, PhD Yoshitane Tsukamoto, MD, PhD An autopsy case of amyloid β-related angiitis with cognitive impairment, multiple infarcts and subcortical hemorrhage Human Pathology: Case Reports |
| title | An autopsy case of amyloid β-related angiitis with cognitive impairment, multiple infarcts and subcortical hemorrhage |
| title_full | An autopsy case of amyloid β-related angiitis with cognitive impairment, multiple infarcts and subcortical hemorrhage |
| title_fullStr | An autopsy case of amyloid β-related angiitis with cognitive impairment, multiple infarcts and subcortical hemorrhage |
| title_full_unstemmed | An autopsy case of amyloid β-related angiitis with cognitive impairment, multiple infarcts and subcortical hemorrhage |
| title_short | An autopsy case of amyloid β-related angiitis with cognitive impairment, multiple infarcts and subcortical hemorrhage |
| title_sort | autopsy case of amyloid β related angiitis with cognitive impairment multiple infarcts and subcortical hemorrhage |
| url | http://www.sciencedirect.com/science/article/pii/S2214330018300865 |
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