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Journal of Neuroinflammation
Published in: Journal of Neuroinflammation 1/2011

Open Access 01-12-2011 | Case report

Cerebral amyloid angiopathy-related inflammation presenting with steroid-responsive higher brain dysfunction: case report and review of the literature

Authors: Hideya Sakaguchi, Akihiko Ueda, Takayuki Kosaka, Satoshi Yamashita, En Kimura, Taro Yamashita, Yasushi Maeda, Teruyuki Hirano, Makoto Uchino

Published in: Journal of Neuroinflammation | Issue 1/2011

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Abstract

A 56-year-old man noticed discomfort in his left lower limb, followed by convulsion and numbness in the same area. Magnetic resonance imaging (MRI) showed white matter lesions in the right parietal lobe accompanied by leptomeningeal or leptomeningeal and cortical post-contrast enhancement along the parietal sulci. The patient also exhibited higher brain dysfunction corresponding with the lesions on MRI. Histological pathology disclosed β-amyloid in the blood vessels and perivascular inflammation, which highlights the diagnosis of cerebral amyloid angiopathy (CAA)-related inflammation. Pulse steroid therapy was so effective that clinical and radiological findings immediately improved.
CAA-related inflammation is a rare disease, defined by the deposition of amyloid proteins within the leptomeningeal and cortical arteries associated with vasculitis or perivasculitis. Here we report a patient with CAA-related inflammation who showed higher brain dysfunction that improved with steroid therapy. In cases with atypical radiological lesions like our case, cerebral biopsy with histological confirmation remains necessary for an accurate diagnosis.
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Literature
1.
Oh U, Gupta R, Krakauer JW, Khandji AG, Chin SS, Elkind MS: Reversible leukoencephalopathy associated with cerebral amyloid angiopathy. Neurology. 2004, 62 (3): 494-497.CrossRefPubMed
2.
Eng JA, Frosch MP, Choi K, Rebeck GW, Greenberg SM: Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol. 2004, 55 (2): 250-256. 10.1002/ana.10810.CrossRefPubMed
3.
Scolding NJ, Joseph F, Kirby PA, Mazanti I, Gray F, Mikol J, Ellison D, Hilton DA, Williams TL, MacKenzie JM, et al: Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Brain. 2005, 128 (Pt 3): 500-515.CrossRefPubMed
4.
Kinnecom C, Lev MH, Wendell L, Smith EE, Rosand J, Frosch MP, Greenberg SM: Course of cerebral amyloid angiopathy-related inflammation. Neurology. 2007, 68 (17): 1411-1416. 10.1212/01.wnl.0000260066.98681.2e.CrossRefPubMed
5.
Nicoll JA, Wilkinson D, Holmes C, Steart P, Markham H, Weller RO: Neuropathology of human Alzheimer disease after immunization with amyloid-beta peptide: a case report. Nat Med. 2003, 9 (4): 448-452. 10.1038/nm840.CrossRefPubMed
6.
Orgogozo JM, Gilman S, Dartigues JF, Laurent B, Puel M, Kirby LC, Jouanny P, Dubois B, Eisner L, Flitman S, et al: Subacute meningoencephalitis in a subset of patients with AD after Abeta42 immunization. Neurology. 2003, 61 (1): 46-54.CrossRefPubMed
7.
Kloppenborg RP, Richard E, Sprengers ME, Troost D, Eikelenboom P, Nederkoorn PJ: Steroid responsive encephalopathy in cerebral amyloid angiopathy: a case report and review of evidence for immunosuppressive treatment. J Neuroinflammation. 2010, 7: 18-10.1186/1742-2094-7-18.PubMedCentralCrossRefPubMed
8.
Schwab P, Lidov HG, Schwartz RB, Anderson RJ: Cerebral amyloid angiopathy associated with primary angiitis of the central nervous system: report of 2 cases and review of the literature. Arthritis Rheum. 2003, 49 (3): 421-427. 10.1002/art.11049.CrossRefPubMed
9.
Greenberg SM, Rapalino O, Frosch MP: Case records of the Massachusetts General Hospital. Case 22-2010. An 87-year-old woman with dementia and a seizure. N Engl J Med. 2010, 363 (4): 373-381. 10.1056/NEJMcpc1004364.CrossRefPubMed
10.
Greenberg SM, Vonsattel JP, Stakes JW, Gruber M, Finklestein SP: The clinical spectrum of cerebral amyloid angiopathy: presentations without lobar hemorrhage. Neurology. 1993, 43 (10): 2073-2079.CrossRefPubMed
11.
Ortiz O, Reed L: Cerebral amyloid angiopathy presenting as a nonhemorrhagic, infiltrating mass. Neuroradiology. 1996, 38 (5): 449-452.PubMed
12.
Fountain NB, Eberhard DA: Primary angiitis of the central nervous system associated with cerebral amyloid angiopathy: report of two cases and review of the literature. Neurology. 1996, 46 (1): 190-197.CrossRefPubMed
13.
Anders KH, Wang ZZ, Kornfeld M, Gray F, Soontornniyomkij V, Reed LA, Hart MN, Menchine M, Secor DL, Vinters HV: Giant cell arteritis in association with cerebral amyloid angiopathy: immunohistochemical and molecular studies. Hum Pathol. 1997, 28 (11): 1237-1246. 10.1016/S0046-8177(97)90196-9.CrossRefPubMed
14.
Fountain NB, Lopes MB: Control of primary angiitis of the CNS associated with cerebral amyloid angiopathy by cyclophosphamide alone. Neurology. 1999, 52 (3): 660-662.CrossRefPubMed
15.
Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 10-2000. A 63-year-old man with changes in behavior and ataxia. N Engl J Med. 2000, 342 (13): 957-965. 10.1056/NEJM200003303421308.
16.
Oide T, Tokuda T, Takei Y, Takahashi H, Ito K, Ikeda S: Serial CT and MRI findings in a patient with isolated angiitis of the central nervous system associated with cerebral amyloid angiopathy. Amyloid. 2002, 9 (4): 256-262. 10.3109/13506120209114103.CrossRefPubMed
17.
Tamargo RJ, Connolly ES, McKhann GM, Khandji A, Chang Y, Libien J, Adams D: Clinicopathological review: primary angiitis of the central nervous system in association with cerebral amyloid angiopathy. Neurosurgery. 2003, 53 (1): 136-143. 10.1227/01.NEU.0000068864.20655.31. discussion 143CrossRefPubMed
18.
Safriel Y, Sze G, Westmark K, Baehring J: MR spectroscopy in the diagnosis of cerebral amyloid angiopathy presenting as a brain tumor. AJNR Am J Neuroradiol. 2004, 25 (10): 1705-1708.PubMed
19.
Hashizume Y, Yoshida M, Suzuki E, Hirayama M: A 65-year-old man with headaches and left homonymous hemianopsia. Neuropathology. 2004, 24 (4): 350-353. 10.1111/j.1440-1789.2004.00588.x.CrossRefPubMed
20.
Harkness KA, Coles A, Pohl U, Xuereb JH, Baron JC, Lennox GG: Rapidly reversible dementia in cerebral amyloid inflammatory vasculopathy. Eur J Neurol. 2004, 11 (1): 59-62. 10.1046/j.1351-5101.2003.00707.x.CrossRefPubMed
21.
Jacobs DA, Liu GT, Nelson PT, Galetta SL: Primary central nervous system angiitis, amyloid angiopathy, and Alzheimer's pathology presenting with Balint's syndrome. Surv Ophthalmol. 2004, 49 (4): 454-459. 10.1016/j.survophthal.2004.04.002.CrossRefPubMed
22.
Mikolaenko I, Conner MG, Jinnah HA: A 50-year-old man with acute-onset generalized seizure. Cerebral amyloid angiopathy and associated giant cell reaction. Arch Pathol Lab Med. 2006, 130 (1): e5-7.PubMed
23.
Wong SH, Robbins PD, Knuckey NW, Kermode AG: Cerebral amyloid angiopathy presenting with vasculitic pathology. J Clin Neurosci. 2006, 13 (2): 291-294. 10.1016/j.jocn.2005.03.025.CrossRefPubMed
24.
Greenberg SM, Parisi JE, Keegan BM: A 63-year-old man with headaches and behavioral deterioration. Neurology. 2007, 68 (10): 782-787. 10.1212/01.wnl.0000258985.31455.13.CrossRefPubMed
25.
Marotti JD, Savitz SI, Kim WK, Williams K, Caplan LR, Joseph JT: Cerebral amyloid angiitis processing to generalized angiitis and leucoencephalitis. Neuropathol Appl Neurobiol. 2007, 33 (4): 475-479. 10.1111/j.1365-2990.2007.00843.x.CrossRefPubMed
26.
McHugh JC, Ryan AM, Lynch T, Dempsey E, Stack J, Farrell MA, Kelly PJ: Steroid-responsive recurrent encephalopathy in a patient with cerebral amyloid angiopathy. Cerebrovasc Dis. 2007, 23 (1): 66-69. 10.1159/000097030.CrossRefPubMed
27.
Takeda A, Tatsumi S, Yamashita M, Yamamoto T: [Granulomatous angiitis of the CNS associated with cerebral amyloid angiopathy--an autopsied case with widespread involvement]. Brain Nerve. 2007, 59 (5): 537-543.PubMed
28.
Machida K, Tojo K, Naito KS, Gono T, Nakata Y, Ikeda S: Cortical petechial hemorrhage, subarachnoid hemorrhage and corticosteroid-responsive leukoencephalopathy in a patient with cerebral amyloid angiopathy. Amyloid. 2008, 15 (1): 60-64. 10.1080/13506120701815589.CrossRefPubMed
29.
Salvarani C, Brown RD, Calamia KT, Christianson TJ, Huston J, Meschia JF, Giannini C, Miller DV, Hunder GG: Primary central nervous system vasculitis: comparison of patients with and without cerebral amyloid angiopathy. Rheumatology (Oxford). 2008, 47 (11): 1671-1677. 10.1093/rheumatology/ken328.CrossRef
30.
Amick A, Joseph J, Silvestri N, Selim M: Amyloid-beta-related angiitis: a rare cause of recurrent transient neurological symptoms. Nat Clin Pract Neurol. 2008, 4 (5): 279-283. 10.1038/ncpneuro0769.CrossRefPubMed
31.
Alcalay RN, Smith EE: MRI showing white matter lesions and multiple lobar microbleeds in a patient with reversible encephalopathy. J Neuroimaging. 2009, 19 (1): 89-91. 10.1111/j.1552-6569.2008.00241.x.CrossRefPubMed
32.
Daniels R, Geurts JJ, Bot JC, Schonewille WJ, van Oosten BW: Steroid-responsive edema in CAA-related inflammation. J Neurol. 2009, 256 (2): 285-286. 10.1007/s00415-009-0136-7.CrossRefPubMed
33.
Morishige M, Abe T, Kamida T, Hikawa T, Fujiki M, Kobayashi H, Okazaki T, Kimura N, Kumamoto T, Yamada A, et al: Cerebral vasculitis associated with amyloid angiopathy: case report. Neurol Med Chir (Tokyo). 2010, 50 (4): 336-338. 10.2176/nmc.50.336.CrossRef
34.
Savoiardo M, Erbetta A, Storchi G, Girotti F: Case 159: cerebral amyloid angiopathy-related inflammation. Radiology. 2010, 256 (1): 323-327. 10.1148/radiol.10091170.CrossRefPubMed
35.
Cano LM, Martinez-Yelamos S, Majos C, Alberti MA, Boluda S, Velasco R, Rubio F: Reversible acute leukoencephalopathy as a form of presentation in cerebral amyloid angiopathy. J Neurol Sci. 2010, 288 (1-2): 190-193. 10.1016/j.jns.2009.09.035.CrossRefPubMed
36.
DiFrancesco JC, Brioschi M, Brighina L, Ruffmann C, Saracchi E, Costantino G, Galimberti G, Conti E, Curto NA, Marzorati L, et al: Anti-Abeta autoantibodies in the CSF of a patient with CAA-related inflammation: a case report. Neurology. 2011, 76 (9): 842-844. 10.1212/WNL.0b013e31820e773c.CrossRefPubMed
37.
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. 2011, 82 (1): 20-26. 10.1136/jnnp.2009.204180.CrossRefPubMed
Metadata
Title
Cerebral amyloid angiopathy-related inflammation presenting with steroid-responsive higher brain dysfunction: case report and review of the literature
Authors
Hideya Sakaguchi
Akihiko Ueda
Takayuki Kosaka
Satoshi Yamashita
En Kimura
Taro Yamashita
Yasushi Maeda
Teruyuki Hirano
Makoto Uchino
Publication date
01-12-2011
Publisher
BioMed Central
Published in
Journal of Neuroinflammation / Issue 1/2011
Electronic ISSN: 1742-2094
DOI
https://doi.org/10.1186/1742-2094-8-116

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