BRAIN and NERVE－神経研究の進歩73巻5号

2021年05月発行

増大特集 中枢神経の自己免疫性・炎症性疾患ハンドブック

第2章　疾患各論

脳アミロイドアンギオパチー関連炎症・血管炎

著者： 坂井健二¹ 山田正仁¹

所属機関： ¹金沢大学大学院医薬保健学総合研究科脳老化・神経病態学（脳神経内科学）

ページ範囲：P.489 - P.495

文献概要

脳アミロイドアンギオパチー関連炎症・血管炎は，脳の皮質・髄膜血管に沈着したアミロイドβ蛋白に対する免疫反応によって炎症が生じたもので，わが国の推定患者数は170人である。急性または亜急性に認知機能障害などを呈し，頭部MRIで出血を伴う白質脳症が見られる。病理では血管周囲へのリンパ球浸潤や多核巨細胞を伴う肉芽腫性血管炎を認める。免疫療法で改善が見込めるため，すみやかな診断および治療開始が推奨される。

参考文献

1）Yamada M: Cerebral amyloid angiopathy: emerging concepts. J Stroke 17: 17-30, 2015

2）Salvarani C, Hunder GG, Morris JM, Brown Jr RD, Christianson T, et al: Aβ-related angiitis: comparison with CAA without inflammation and primary CNS vasculitis. Neurology 81: 1596-1603, 2013

3）Yamada M, Tsukagoshi H, Otomo E, Hayakawa M: Cerebral amyloid angiopathy in the aged. J Neurol 234: 371-376, 1987

4）Sakai K, Ueda M, Fukushima W, Tamaoka A, Shoji M, et al: Nationwide survey on cerebral amyloid angiopathy in Japan. Eur J Neurol 26: 1487-1493, 2019

5）Corovic A, Kelly S, Markus HS: Cerebral amyloid angiopathy associated with inflammation: a systematic review of clinical and imaging features and outcome. Int J Stroke 13: 257-267, 2018

6）Kinnecom C, Lev MH, Wendell L, Smith EE, Rosand J, et al: Course of cerebral amyloid angiopathy- related inflammation. Neurology 68: 1411-1416, 2007

7）Albargothy NJ, Johnston DA, MacGregor-Sharp M, Weller RO, Verma A, et al: Convective influx/glymphatic system: tracers injected into the CSF enter and leave the brain along separate periarterial basement membrane pathways. Acta Neuropathol 136: 139-152, 2018

8）Yamada M, Itoh Y, Shintaku M, Kawamura J, Jensson O, et al: Immune reactions associated with cerebral amyloid angiopathy. Stroke 27: 1155-1162, 1996

9）Scolding NJ, Joseph F, Kirby PA, Mazanti I, Gray F, et al: Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain 128: 500-515, 2005

10）Sakai K, Hayashi S, Sanpei K, Yamada M, Takahashi H: Multiple cerebral infarcts with a few vasculitic lesions in the chronic stage of cerebral amyloid angiopathy-related inflammation. Neuropathology 32: 551-556, 2012

11）Piazza F, Greenberg SM, Savoiardo M, Gardinetti M, Chiapparini L, et al: Anti-amyloid β autoantibodies in cerebral amyloid angiopathy-related inflammation: implications for amyloid-modifying therapies. Ann Neurol 73: 449-458, 2013

12）Kimura A, Sakurai T, Yoshikura N, Hayashi Y, Takemura M, et al: Corticosteroid therapy in a patient with cerebral amyloid angiopathy-related inflammation. J Neuroinflammation 10: 39, 2013［doi: 10.1186/1742-2094-10-39］

13）Sakai K, Noguchi-Shinohara M, Ikeda T, Hamaguchi T, Ono K, et al: Cerebrospinal fluid cytokines and metalloproteinases in cerebral amyloid angiopathy-related inflammation. Acta Neurol Scand 143: 450-457, 2021

14）Chung KK, Anderson NE, Hutchinson D, Synek B, Barber PA: Cerebral amyloid angiopathy related inflammation: three case reports and a review. J Neurol Neurosurg Psychiatry 82: 20-26, 2011

15）DiFrancesco JC, Brioschi M, Brighina L, Ruffmann C, Saracchi E, et al: Anti-Aβ autoantibodies in the CSF of a patient with CAA-related infllammation: a case report. Neurology 76: 842-844, 2011

16）Martucci M, Sarria S, Toledo M, Coscojuela P, Vert C, et al: Cerebral amyloid angiopathy-related inflammation: imaging findings and clinical outcome. Neuroradiology 56: 283-289, 2014

17）Johnson KA, Gregas M, Becker JA, Kinnecom C, Salat DH, et al: Imaging of amyloid burden and distribution in cerebral amyloid angiopathy. Ann Neurol 62: 229-234, 2007

18）Miller DV, Salvarani C, Hunder GG, Brown RD, Parisi JE, et al: Biopsy findings in primary angiitis of the central nervous system. Am J Surg Pathol 33: 35-43, 2009

19）Knudsen KA, Rosand J, Karluk D, Greenberg SM: Clinical diagnosis of cerebral amyloid angiopathy: validation of the Boston criteria. Neurology 56: 537-539, 2001

20）Charidimou A, Gang Q, Werring DJ: Sporadic cerebral amyloid angiopathy revisited: recent insights into pathophysiology and clinical spectrum. J Neurol Neurosurg Psychiatry 83: 124-137, 2012

21）Auriel E, Charidimou A, Gurol ME, Ni J, Van Etten ES, et al: Validation of clinicoradiological criteria for the diagnosis of cerebral amyloid angiopathy-related inflammation. JAMA Neurol 73: 197-202, 2016

22）Dutra LA, de Souza AWS, Grinberg-Dias G, Barsottini OGP, Appenzeller S: central nervous system vasculitis in adults: an update. Autoimmun Rev 16: 123-131, 2017

23）Regenhardt RW, Thon JM, Das AS, Thon OR, Charidimou A, et al: Association between immunosuppressive treatment and outcomes of cerebral amyloid angiopathy-eelated inflammation. JAMA Neurol 77: 1-10, 2020

24）Hashizume Y, Yoshida M, Suzuki E, Hirayama M: A 65-year-old man with headaches and left homonymous hemianopsia. Neuropathology 24: 350-353, 2004