出血性病変が検出されずに広範な脳病変に至ったアミロイドβ関連血管炎の1例
アミロイドβ関連血管炎（amyloid β-related angiitis）では，皮質や皮質下の微小出血や脳表ヘモジデリンの沈着が，脳MRI上の重要な所見である．我々は，治療前にはこれらの所見を呈さなかったアミロイドβ関連血管炎を提示する．症例は75歳女性．右同名半盲と失語で発症し，昏睡に至った．脳MRIでは，びまん性の軟髄膜造影病変と散在性のDWI高信号病変を認めたが，T2*WIでは微小出血は検出されなかった．病理所見からアミロイドβ関連血管炎と診断した．ステロイド治療により画像所見，臨床症状ともに改善した．治療後のsusceptibility-weighted imaging（SWI）で...
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| Published in | 臨床神経学 Vol. 60; no. 3; pp. 187 - 192 |
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| Main Authors | , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
日本神経学会
2020
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0009-918X 1882-0654 |
| DOI | 10.5692/clinicalneurol.cn-001340 |
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| Abstract | アミロイドβ関連血管炎（amyloid β-related angiitis）では，皮質や皮質下の微小出血や脳表ヘモジデリンの沈着が，脳MRI上の重要な所見である．我々は，治療前にはこれらの所見を呈さなかったアミロイドβ関連血管炎を提示する．症例は75歳女性．右同名半盲と失語で発症し，昏睡に至った．脳MRIでは，びまん性の軟髄膜造影病変と散在性のDWI高信号病変を認めたが，T2*WIでは微小出血は検出されなかった．病理所見からアミロイドβ関連血管炎と診断した．ステロイド治療により画像所見，臨床症状ともに改善した．治療後のsusceptibility-weighted imaging（SWI）では多数の微小出血を認めた．アミロイドβ関連血管炎の非侵襲的診断のために，微小出血以外の画像の特徴を集積すべきである． |
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| AbstractList | アミロイドβ関連血管炎（amyloid β-related angiitis）では，皮質や皮質下の微小出血や脳表ヘモジデリンの沈着が，脳MRI上の重要な所見である．我々は，治療前にはこれらの所見を呈さなかったアミロイドβ関連血管炎を提示する．症例は75歳女性．右同名半盲と失語で発症し，昏睡に至った．脳MRIでは，びまん性の軟髄膜造影病変と散在性のDWI高信号病変を認めたが，T2*WIでは微小出血は検出されなかった．病理所見からアミロイドβ関連血管炎と診断した．ステロイド治療により画像所見，臨床症状ともに改善した．治療後のsusceptibility-weighted imaging（SWI）では多数の微小出血を認めた．アミロイドβ関連血管炎の非侵襲的診断のために，微小出血以外の画像の特徴を集積すべきである． |
| Author | 山岸, 拓磨 畠野, 雄也 柿田, 明美 中島, 章博 須貝, 章弘 小野寺, 理 |
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| References | 13) Salvarani C, Morris JM, Giannini C, et al. Imaging findings of cerebral amyloid angiopathy, Aβ-related angiitis (ABRA), and cerebral amyloid angiopathy–related inflammation. Medicine (Baltimore) 2016;95:e3613. 4) Chung KK, Anderson NE, Hutchinson D, et al. Cerebral amyloid angiopathy related inflammation: three case reports and a review. J Neurol Neurosurg Psychiatry 2011;82:20-26. 10) Kurian M, Burkhardt K, Assal F, et al. Amyloid plaques and intraneuronal tau inclusions in A-beta-related angiitis (ABRA). Neuropathol Appl Neurobiol 2012;38:391-394. 5) Auriel E, Charidimou A, Gurol ME, et al. Validation of clinicoradiological criteria for the diagnosis of cerebral amyloid angiopathy–related inflammation. JAMA Neurol 2016;73:197-202. 7) Charidimou A, Gang Q, Werring DJ. Sporadic cerebral amyloid angiopathy revisited: recent insights into pathophysiology and clinical spectrum. J Neurol Neurosurg Psychiatry 2012;83:124-137. 14) 小池佑佳，大内東香，佐藤朋江ら．MRIで髄膜造影を呈し脳生検で診断した脳アミロイドβ関連血管炎の1例．Brain Nerve 2013;65:693-697. 15) Martucci M, Sarria S, Toledo M, et al. Cerebral amyloid angiopathy-related inflammation: imaging findings and clinical outcome. Neuroradiology 2014;56:283-289. 11) Cheng AL, Batool S, McCreary CR, et al. Susceptibility-weighted imaging is more reliable than T2*-weighted gradient-recalled echo MRI for detecting microbleeds. Stroke 2013;44: 2782-2786. 6) Sakaguchi H, Ueda A, Kosaka T, et al. Cerebral amyloid angiopathy-related inflammation presenting with steroid-responsive higher brain dysfunction: case report and review of the literature. J Neuroinflammation 2011;8:116. 12) Salvarani C, Hunder GG, Morris JM, et al. Aβ-related angiitis: comparison with CAA without inflammation and primary CNS vasculitis. Neurology 2013;81:1596-1603. 3) Danve A, Grafe M, Deodhar A. Amyloid beta-related angiitis—a case report and comprehensive review of literature of 94 cases. Semin Arthritis Rheum. 2014;44:86-92. 2) Eng JA, Frosch MP, Choi K, et al. Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol 2004;55:250-256. 1) Moussaddy A, Levy A, Strbian D, et al. Inflammatory cerebral amyloid angiopathy, amyloid-β–related angiitis, and primary angiitis of the central nervous system: similarities and differences. Stroke 2015;46:e210-e213. 9) Scolding NJ, Joseph F, Kirby PA, et al. Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain 2005;128:500-515. 8) Charidimou A, Krishnan A, Werring DJ, et al. Cerebral microbleeds: a guide to detection and clinical relevance in different disease settings. Neuroradiology 2013;55:655-674. |
| References_xml | – reference: 10) Kurian M, Burkhardt K, Assal F, et al. Amyloid plaques and intraneuronal tau inclusions in A-beta-related angiitis (ABRA). Neuropathol Appl Neurobiol 2012;38:391-394. – reference: 4) Chung KK, Anderson NE, Hutchinson D, et al. Cerebral amyloid angiopathy related inflammation: three case reports and a review. J Neurol Neurosurg Psychiatry 2011;82:20-26. – reference: 1) Moussaddy A, Levy A, Strbian D, et al. Inflammatory cerebral amyloid angiopathy, amyloid-β–related angiitis, and primary angiitis of the central nervous system: similarities and differences. Stroke 2015;46:e210-e213. – reference: 12) Salvarani C, Hunder GG, Morris JM, et al. Aβ-related angiitis: comparison with CAA without inflammation and primary CNS vasculitis. Neurology 2013;81:1596-1603. – reference: 3) Danve A, Grafe M, Deodhar A. Amyloid beta-related angiitis—a case report and comprehensive review of literature of 94 cases. Semin Arthritis Rheum. 2014;44:86-92. – reference: 7) Charidimou A, Gang Q, Werring DJ. Sporadic cerebral amyloid angiopathy revisited: recent insights into pathophysiology and clinical spectrum. J Neurol Neurosurg Psychiatry 2012;83:124-137. – reference: 9) Scolding NJ, Joseph F, Kirby PA, et al. Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain 2005;128:500-515. – reference: 11) Cheng AL, Batool S, McCreary CR, et al. Susceptibility-weighted imaging is more reliable than T2*-weighted gradient-recalled echo MRI for detecting microbleeds. Stroke 2013;44: 2782-2786. – reference: 13) Salvarani C, Morris JM, Giannini C, et al. Imaging findings of cerebral amyloid angiopathy, Aβ-related angiitis (ABRA), and cerebral amyloid angiopathy–related inflammation. Medicine (Baltimore) 2016;95:e3613. – reference: 14) 小池佑佳，大内東香，佐藤朋江ら．MRIで髄膜造影を呈し脳生検で診断した脳アミロイドβ関連血管炎の1例．Brain Nerve 2013;65:693-697. – reference: 6) Sakaguchi H, Ueda A, Kosaka T, et al. Cerebral amyloid angiopathy-related inflammation presenting with steroid-responsive higher brain dysfunction: case report and review of the literature. J Neuroinflammation 2011;8:116. – reference: 8) Charidimou A, Krishnan A, Werring DJ, et al. Cerebral microbleeds: a guide to detection and clinical relevance in different disease settings. Neuroradiology 2013;55:655-674. – reference: 15) Martucci M, Sarria S, Toledo M, et al. Cerebral amyloid angiopathy-related inflammation: imaging findings and clinical outcome. Neuroradiology 2014;56:283-289. – reference: 2) Eng JA, Frosch MP, Choi K, et al. Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol 2004;55:250-256. – reference: 5) Auriel E, Charidimou A, Gurol ME, et al. Validation of clinicoradiological criteria for the diagnosis of cerebral amyloid angiopathy–related inflammation. JAMA Neurol 2016;73:197-202. |
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| Title | 出血性病変が検出されずに広範な脳病変に至ったアミロイドβ関連血管炎の1例 |
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