[18] It can be concluded that these pathologically similar diseases constitute a spectrum from CAA to PACNS [Table 1]. Other diagnostic indexes include the apolipoprotein E 4 allele, A and anti-A antibodies in cerebral spinal fluid and amyloid positron emission tomography. [16,17] However, the terms used to describe this disease are confusing. Brain MRI lesions; Cerebral amyloid angiopathy; Cerebral small vessel disease; Inflammation; Review. Immunosuppressive therapy is effective both during initial presentation and in relapses. Cerebral amyloid angiopathy and cerebral amyloid angiopathy-related inflammation: comparison of hemorrhagic and DWI MRI features. Cerebral amyloid angiopathy-related inflammation (CAA-ri), also referred to as inflammatory cerebral amyloid angiopathy and A-related angiitis, is a distinct subset of cerebral amyloid angiopathy (CAA) characterized by an autoimmune reaction to cerebrovascular -amyloid deposits. sharing sensitive information, make sure youre on a federal 1 Introduction of the imaging-based Boston criteria for diagnosis of CAA in the 1990s 2, 3 [62,63] Thus, it is very important to recognize the clinical and radiological properties of CAA-RI and bear some differential diagnoses in mind; those substantial differential diagnoses should be ruled out before CAA-RI was diagnosed. Brain MRI 9 months later showed multiple discrete regions . 61. This article reviews the pathology and pathogenesis, clinical and imaging manifestations, diagnostic criteria, treatment, and prognosis of CAA-RI, and highlights unsolved problems in the existing research. Semin Arthritis Rheum. CAA can present on imaging as CAA (common), amyloidoma (uncommon), or inflammatory CAA (rare). [14] In addition to A deposition, CAA-RI also demonstrates pronounced perivascular or transmural inflammatory infiltration. 31. The growing clinical spectrum of cerebral amyloid angiopathy. Clinical manifestations of cerebral amyloid angiopathy-related inflammation. An official website of the United States government. Cerebral amyloid angiopathy-related inflammation (CAA-ri) is characterized by sub-acute confusion, progressive cognitive decline, seizure or headaches; reversible focal subcortical and/or cortical T2 hyperintensities on magnetic resonance imaging (MRI); and neuropathological evidence of cerebral amyloid angiopathy (CAA) and associated vascular or perivascular inflammation [1-3]. Similar clinical processes and radiological changes of CAA-RI appear in amyloid-related imaging abnormalities (ARIA), initially during the clinical trial of bapineuzumab, the monoclonal antibody for AD, and later in that of other amyloid modification therapies. [28] CAA-RI is thought to be a spontaneous ARIA, while ARIA is considered to be iatrogenic CAA-RI. [69] A systematic review of both pathological subtypes revealed that, during an average follow-up period of 24 months, 55% of patients eventually end up being asymptomatic or with mild disability. [46] Two-thirds of ABRA patients and only 31.3% of ICAA patients showed contrast enhancement on MRI. [17] And the youngest case with pathologic evidence ever reported was 42 years old.[34]. [3] CAA related lobar ICH has been identified as the second most common form of spontaneous ICH following hypertensive angiopathy. 7. Inflammatory cerebral amyloid angiopathy is an uncommon cerebral amyloid deposition disease, closely related to the far more common non-inflammatory cerebral amyloid angiopathy , and can present as areas of vasogenic edema. Medicina (Kaunas). 53. Yamada M. Cerebral amyloid angiopathy: emerging concepts. [11] The most commonly used immunosuppressants are cyclophosphamide (33.9%), azathioprine (5.0%), mycophenolate mofetil (5.0%), methotrexate, immunoglobulin, and so on. 36. 12. [20] Currently, most evidence favors the hypothesis that inflammation is triggered by an autoimmune response to the deposited A protein. Probable Cerebral Amyloid Angiopathy-Related Inflammation Associated With Sitravatinib: A Case Report. Thus, other differential diagnoses should be carefully ruled out. 2016 May;95(20):e3613. Inflammatory Cerebral Amyloid Angiopathy, Amyloid-Related Angiitis, and Primary Angiitis of the Central Nervous System. Amyloid can be confirmed when the Congo red-stained section shows green birefringence under polarized light. Copyright 2021 Elsevier B.V. All rights reserved. Growing numbers of patients have been reported with vascular inflammation associated with advanced cerebral amyloid angiopathy (59; 150). [33] Findings from several systematic reviews have shown that there is no obvious gender difference, but a slight male predominance was observed. CAA-RI shares pathologic characteristics of CAA, which is A deposition in the cortical or leptomeningeal vessels, with positive Congo red staining. Cerebral amyloid angiopathy (CAA)related inflammation (CAA-RI) affects brain parenchyma, but rarely involves leptomeninges, a likely immunogenic consequence of -amyloid peptide expressed in the walls of small and medium sized cerebral vessels. Cancelloni V, Rufa A, Battisti C, De Stefano N, Mastrocinque E, Garosi G, Venezia D, Chiarotti I, Cerase A. Neurol Sci. Once the diagnosis is made, glucocorticoids or even immunosuppressants should be adopted in order to improve the prognosis. Tumoral presentation of homonymous hemianopia and prosopagnosia in cerebral amyloid angiopathy-related inflammation. (2016) Neurology. Summary of MRI markers of small vessel disease and CAA to be evaluated in the project, including their definition, ratings scales and important points/modifications in their assessment specifically for clinical use within the Boston criteria v.2.0. The diagnostic criteria for "probable" inflammatory cerebral amyloid angiopathy require white matter hyperintensities on T2-FLAIRthat are asymmetric and extend to the immediately subcortical white matter 4. [11] The gold standard test for diagnosis is autopsy or brain biopsy. Aghetti A, Sene D, Polivka M, Shor N, Lechtman S, Chabriat H, et al. Thirteen percent of patients were affected with some forms of visual impairment. Melzer N, Harder A, Gross CC, Wolfer J, Stummer W, Niederstadt T, et al. Early diagnosis and timely treatment may improve prognosis. 54. [13] For patients diagnosed with probable CAA-RI by means of these criteria, immunosuppressive therapy can be given empirically to avoid brain biopsy. Stroke 2014; 45:26362642. After treatment with corticoids, (D) WMH faded significantly. An increase in inflammatory biomarkers has been observed in CAA-RI patients in different studies. Amyloid beta-related angiitis--a case report and comprehensive review of literature of 94 cases. Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy. Aghetti A, Sne D, Polivka M, Shor N, Lechtman S, Chabriat H, Jouvent E, Guey S. Cerebral Amyloid Angiopathy Related Inflammation With Prominent Meningeal Involvement. In patients who respond to treatment, imaging follow-up demonstrates regression of the aforementioned inflammatory findings. Nouh A, Borys E, Gierut AK, Biller J. Amyloid-Beta related angiitis of the central nervous system: case report and topic. Get new journal Tables of Contents sent right to your email inbox, http://creativecommons.org/licenses/by-nc-nd/4.0, Cerebral amyloid angiopathy-related inflammation: current status and future implications, Articles in Google Scholar by Juan-Juan Wu, Other articles in this journal by Juan-Juan Wu, China Association for Science and Technology, Chinese Medical Association (Sponsor of CMJ), Chinese Medical Association Publishing House, International Committee of Medical Journal Editors, Privacy Policy (Updated December 15, 2022). Saliou V, Ben Salem D, Ognard J, Guellec D, Marcorelles P, Rouhart F, et al. There are two major types of CAA: one is hereditary CAA, which is associated with Down syndrome or mutations in the A protein precursor (APP) gene or presenilin gene,[1] and the other one is age-related sporadic CAA. In contrast to CAA, which is currently without effective treatment, most studies have shown that empirical high-dose corticosteroids with or without additional immunosuppressive therapy can mitigate symptoms and imaging abnormalities and can improve the prognosis of CAA-RI. 15 (8): 54. However, the prognosis of most untreated patients is poor. (B) Strictly lobar, MeSH A Collet-Sicard syndrome due to internal carotid artery dissection associated with cerebral amyloid angiopathy-related inflammation. Sallles E, Bonneville F, Delisle MB, Rigal E, Raposo N, Pariente J. It would be more difficult to identify patients who also have a history of tumors. Mandal J, Chung SA. Unauthorized use of these marks is strictly prohibited. -, Yeh SJ, Tang SC, Tsai LK, Jeng JS. 2020; 16:30-42. doi: 10.1038/s41582-019-0281-2 Google Scholar; 35. [17,18] The main patient group is the elderly, with an average age of 67 at diagnosis; yet, this is still younger than that of CAA patients. The diagnostic criteria for both probable and possible inflammatory cerebral amyloid angiopathy require at least one corticosubcortical hemorrhagic lesion 4, which is best demonstrated as signal loss on T2*-weighted sequences (susceptibility-weightedor gradient echo): cerebral macrobleed (intraparenchymal hematoma), cerebral microbleed (cerebral microhemorrhage). (2013) American Journal of Neuroradiology. Miller-Thomas MM, Sipe AL, Benzinger TL et-al. Table 3. CT and MRI demonstrate an area of vasogenic edema involving the subcortical white matter 1. 8600 Rockville Pike Amyloid-Beta Related Angitiis and Reversible Cerebral Vasoconstriction Syndrome: A Case Report (P6.057). The diagnostic efficiency for possible CAA-RI is low, with a specificity of only 68%. Typical images of cerebral amyloid angiopathy-related inflammation. 33. Recurrence of cerebral amyloid angiopathy-related inflammation: a report of two cases from the iCAbeta international network. Cerebral Amyloid Angiopathy (CAA) Associated with Inflammation (Inflammatory CAA) Background: Cerebral amyloid angiopathy (CAA) is characterized by deposition of amyloid-beta in the media and adventitia of cortical and leptomeningeal arteries. A Report of 2 Cases. 11. The gold standard for diagnosis is autopsy or brain biopsy. 12. The use of glucocorticoids and immunosuppressants improves prognosis. 72. Please try again soon. Acta Neuropathol 1974; 27:131137. Fukasawa R, Shimizu S, Hirose D, Kanetaka H, Umahara T, Obikane H, et al. Clipboard, Search History, and several other advanced features are temporarily unavailable. Epub 2019 May 25. Terminology Second, vasculitis and the vascular areas affected by A co-localize. Salloway SP, Sperling R, Fox NC, Sabbagh MN, Honig LS, Porsteinsson AP, et al. The resultant vascular fragility tends to manifest in normotensive elderly patients as lobar intracerebral hemorrhage. 47. [22] Nevertheless, in our experience, this is not typical and may not be meaningful in clinical practice. 22. [48,49], Gadolinium enhancement of parenchyma or leptomeninges may or may not be present [Figure 1],[43,50] although the proportion of enhancing cases in CAA-RI is significantly higher than that in non-inflammatory CAA cases. Correspondence to: Dr. Jun Ni, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, No 1, Shuaifuyuan, Dongdan, Dongcheng District, Beijing 10073, ChinaE-Mail: [emailprotected], How to cite this article: Wu JJ, Yao M, Ni J. Cerebral amyloid angiopathy-related inflammation: current status and future implications. Cases of an isolated leptomeningeal process on imaging are more commonly categorized as amyloid related angiitis, within the limitations of variable terminology noted above 6. [17] Steroid therapy is also effective during recurrence, but increased microbleeds may be detected with T2/SWI sequences in that case. Salvarani C, Morris JM, Giannini C, Brown RD Jr, Christianson T, Hunder GG. Salvarani C, Morris JM, Giannini C, Brown RD Jr, Christianson T, Hunder GG. Cerebrospinal fluid anti-amyloid- autoantibodies and amyloid PET in cerebral amyloid angiopathy-related inflammation. The .gov means its official. Child ND, Braksick SA, Flanagan EP, Keegan BM, Giannini C, Kantarci OH. 71. Sengoku R, Matsushima S, Murakami Y, Fukuda T, Tokumaru AM, Hashimoto M, et al. (B) Strictly lobar CMBs. After several recurrences, WMH and CMBs progressed and long-term follow-up led to a diagnosis of CAA-RI. Shams S, Martola J, Cavallin L, Granberg T, Shams M, Aspelin P, et al. Cerebral amyloid angiopathy-related inflammation in the immunosuppressed: a case report. A case of cerebral amyloid angiopathy-related inflammation with the rare apolipoprotein epsilon2/epsilon2 genotype. Salvarani C, Brown RD Jr, Calamia KT, Christianson TJ, Huston J 3rd, Meschia JF, et al. A spectrum from CAA to PACNS: pathological differences between CAA, ICAA, ABRA, and PACNS. Highlight selected keywords in the article text. [32] In a systematic review, of the 142 cases with available data, 27.5% presented with both perivascular inflammation and vasculitis with granuloma formation, which is the most common pathological pattern. Bogner S, Bernreuther C, Matschke J, Barrera-Ocampo A, Sepulveda-Falla D, Leypoldt F, et al. Anti-A autoantibodies in the CSF of a patient with CAA-related inflammation: a case report. 37. [11] This phenomenon may be explained by the fact that the blood vessel wall in cases of ICAA is less destroyed than that in cases of ABRA. It is worth noting that CAA-RI is a diagnosis by exclusion. [18] Sakai et al[32] reported a case of CAA-RI at the chronic stage, with persistently elevated proteinase 3-antineutrophil cytoplasmic antibody levels. The Karolinska Imaging Dementia Study. Cerebral amyloid angiopathy (CAA) is an important cause of cognitive impairment and spontaneous intracerebral hemorrhage in the elderly. Radiographics. 8. Anti-amyloid beta autoantibodies in cerebral amyloid angiopathy-related inflammation: implications for amyloid-modifying therapies. Giant cell arteritis and arteriolitis associated with amyloid angiopathy in an elderly mongol. 1. Cancelloni V, Rufa A, Battisti C, De Stefano N, Mastrocinque E, Garosi G, Venezia D, Chiarotti I, Cerase A. Neurol Sci. In addition, CAA is a disease caused by disordered A clearance, and CAA-RI is in fact the body's immune response aimed at clearing A. Nat Rev Neurol. Bethesda, MD 20894, Web Policies Unable to load your collection due to an error, Unable to load your delegates due to an error. 6. -. 51 (2): 525-32. You may be trying to access this site from a secured browser on the server. SWI or T2: which MRI sequence to use in the detection of cerebral microbleeds? DiFrancesco JC, Longoni M, Piazza F. Anti-Abeta autoantibodies in amyloid related imaging abnormalities (ARIA): candidate biomarker for immunotherapy in Alzheimer's disease and cerebral amyloid angiopathy. Leptomeningeal enhancement may be a unique imaging manifestation in some cases with confirmed CAA-RI. 5. 280 (2): 643-7. 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. However, biopsy is invasive; consequently, most clinically diagnosed cases have been based on clinical and radiological data. official website and that any information you provide is encrypted Medicine (Baltimore). Brashear, H.M. Arrighi, K.A. Subcortical white matter will demonstrate usually a solitary area of low density with localized mass effect 1,2. Cerebral Amyloid Angiopathy (CAA)-Related Inflammation: Comparison of Inflammatory CAA and Amyloid--Related Angiitis. MRI is the modality of choice in assessing these patients as it is able to visualize the characteristic peripheral microhemorrhages of cerebral amyloid angiopathy. Primary angiitis of the central nervous system. Immunosuppressive therapy is effective both during initial presentation and in relapses. Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare but increasingly recognized subtype of CAA. Kang P, Bucelli RC, Ferguson CJ, Corbo JC, Kim AH, Day GS. Acta Neuropathol. Revesz T, Holton JL, Lashley T, Plant G, Frangione B, Rostagno A, Ghiso J. Genetics and molecular pathogenesis of sporadic and hereditary cerebral amyloid angiopathies. Auriel et al[13] updated the criteria in 2016, defined the WMH pattern specific for distinguishing between probable and possible CAA-RI, and proposed cSS as a marker of hemorrhage. Amyloid--related angiitis: a report of 2 cases with unusual presentations. [2] CAA is clinically diverse. Although originally defined as a clinicopathologic diagnosis, it can now often be diagnosed based on clinicoradiologic criteria, though confirmation with brain and meningeal biopsy is still required in some cases. Coulette S, Renard D, Lehmann S, Raposo N, Arquizan C, Charif M, et al. [14], Angio-destructive changes, such as fibrinoid necrosis can also be found in some of the vessel walls in patients affected by ABRA. Copyright 2021 The Chinese Medical Association, produced by Wolters Kluwer, Inc. under the CC-BY-NC-ND license. Renard D, Tatu L, Collombier L, Wacongne A, Ayrignac X, Charif M, Boukriche Y, Chiper L, Fourcade G, Azakri S, Gaillard N, Mercier E, Lehmann S, Thouvenot E. J Alzheimers Dis. Pathological changes within the cerebral vasculature in Alzheimer's disease: New perspectives. Primary central nervous system vasculitis: comparison of patients with and without cerebral amyloid angiopathy. Hemorrhage and white matter injury seen at imaging reflect vascular damage caused by the accumulation of A in vessel walls. [2,46,68] The most common abnormality found in PACNS is the presence of proximal or distal stenosis on MRA or conventional digital subtraction angiography; this is not commonly seen in CAA-RI. Neuroradiology. Szpak GM, Lewandowska E, Sliwiska A, Stpie T, Tarka S, Mendel T, et al. 35. There are still many questions related to CAA-RI that require investigation. AD patients who are apolipoprotein E (APOE) 4 gene carriers are more likely to develop ARIA after anti-A treatment,[25,26] in accordance with the findings in CAA-RI. [12,13] Because immunosuppressive therapy is effective for the disease, timely diagnosis and early commencement of therapy are very important. There is currently no study giving recommendations on the choice of medication, dosage, and the time span of treatment. 40. Some authors are consistent with the terms we have used here, while some call the two subtypes CAA-RI and ABRA. DiFrancesco JC, Brioschi M, Brighina L, Ruffmann C, Saracchi E, Costantino G, et al. 38. 69. Perivascular and vascular inflammatory patterns without granulomas accounted for 22.5% of cases. 23. Since there is no A deposition in the blood vessels supplying the spinal cord, symptoms of myelopathy have not been reported in ICAA and ABRA; thus, PACNS is a more likely diagnosis when symptoms involving the spinal cord occur. HHS Vulnerability Disclosure, Help Beta-APP42 may activate mononuclear phagocytes in the brain and elicit inflammatory responses. However, some studies have questioned the idea. Keyword Highlighting J Alzheimers Dis. Theodorou A, Palaiodimou L, Safouris A, Kargiotis O, Psychogios K, Kotsali-Peteinelli V, Foska A, Zouvelou V, Tzavellas E, Tzanetakos D, Zompola C, Tzartos JS, Voumvourakis K, Paraskevas GP, Tsivgoulis G. J Clin Med. Yeh SJ, Tang SC, Tsai LK, Jeng JS. Cerebral amyloid--related angiitis without cerebral microbleeds in a patient with subarachnoid hemorrhage. Long-term follow up of patients with mild-to-moderate Alzheimer's disease treated with bapineuzumab in a phase III, open-label, extension study. Other differential diagnoses include viral or autoimmune encephalitis, cerebral venous thrombosis, acute disseminated encephalomyelitis (ADEM), Hashimoto encephalopathy, neurosarcoidosis, and acute toxic-metabolic leukoencephalopathy. American journal of neuroradiology. The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). This pathological distinction is not reliably predicted on imaging 2. [14] However, findings from another study have suggested that non-specific vascular changes in ABRA may be observed when medium-sized arteries are involved. Moosavi B, Torres C, Jansen G. Case 232: Amyloid -related Angiitis. Moreover, the efficacy of treatment was evaluated by observational studies; consequently, more clinical trials and even randomized clinical trials are required. 43. In order to make a diagnosis before histopathology, Chung et al[12] proposed the Boston criteria using clinicoradiological data in 2011. 256 (1): 323-7. Acute or subacute onset of cognitive decline or behavioral changes is the most common symptom of CAA-RI. The distribution of CMBs does not follow the regional pattern of occipital dominance in non-inflammatory CAA. 56. 66. 58. -, Wermer MJH, Greenberg SM. 24. Porter M, Newey CR, Toth G. Teaching NeuroImages: treatment-resistant rapidly progressive amyloid -related angiitis. Zhu X, Schrader JM, Irizarry BA, Smith SO, Van Nostrand WE. Some of these diseases can be ruled out by T2 MRI or SWI. Piazza F, Greenberg SM, Savoiardo M, Gardinetti M, Chiapparini L, Raicher I, et al. [22,31] In fact, both ICAA and ABRA can present with or without granulomatous inflammation. 2022 Dec 3;22(1):449. doi: 10.1186/s12883-022-02979-6. Prodromal Alzheimer's disease presenting as cerebral amyloid angiopathy-related inflammation with spontaneous amyloid-related imaging abnormalities and high cerebrospinal fluid anti-A autoantibodies. The white matter hyperintensity represents vasogenic edema, which may show localized mass effect. - "Advancing diagnostic criteria for sporadic cerebral amyloid angiopathy: Study protocol for a multicenter MRI-pathology validation of Boston criteria v2.0" Some cases presented with involuntary movement,[35,36] while others had systemic diseases,[14] cerebral hernia caused by severe edema,[37] uveitis,[21] multiple malignancies,[14,15,38] extracranial vasculitis, or vascular dysplasia at baseline. Sugihara S, Ogawa A, Nakazato Y, Yamaguchi H. Cerebral beta amyloid deposition in patients with malignant neoplasms: its prevalence with aging and effects of radiation therapy on vascular amyloid. Occasional cases of pathologically-confirmed inflammatory cerebral amyloid angiopathy have been reported with prominent leptomeningeal involvement without the typical white matter or hemorrhagic lesions on imaging 5,6. Neurol Clin Pract. Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare variant of CAA with autoimmune inflammation. Both variants produce a clinical picture that resembles primary angiitis of the CNS but is distinguished by a characteristic radiologic appearance. [72] It is worth noting that this case involved a patient who had been using immunosuppressive agents. (C) No enhancement was seen. [44,45] However, sometimes the burden of CMBs is so obvious that hypointense lesions seen on SWI can also be identified on T2 or FLAIR images. Yamada M. Cerebral amyloid angiopathy: emerging concepts. doi: 10.1212/WNL.0b013e3182a9f545. Ronsin S, Deiana G, Geraldo AF, Durand-Dubief F, Thomas-Maisonneuve L, Formaglio M, et al. Diagnosis, treatment, and follow-up of patients with cerebral amyloid angiopathy-related inflammation. There are two recognized pathologically characterized variants: cerebral amyloid angiopathy-related inflammation (CAAri) and A beta-related angiitis (ABRA). Department of Neurology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China. Cerebral amyloid angiopathy (CAA) is a kind of disease in which amyloid (A) and other amyloid protein deposits in the cerebral cortex and the small blood vessels of the brain, causing . Please enable scripts and reload this page. Objective. However, many patients present with atypical symptoms other than those mentioned above, which may easily lead to an incorrect diagnosis. 6. In the remainder, which accounts for 60% of all affected individuals, even with treatment severe disability or death are encountered 2. However, the average patient is a little younger than in non-inflammatory cerebral amyloid angiopathy and older than those with non-amyloid primary cerebral angiitis 2. (A) Confluent WMH. CAARI, also called amyloid--related angiitis, is a rare form of cerebral amyloid angiopathy with a predominantly vascular inflammation or angiitis. Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare but increasingly recognized subtype of CAA. An alternative transcript of the Alzheimer's disease risk gene SORL1 encodes a truncated receptor. FOIA MRA and vessel wall imaging may show medium-sized arteries involved with multifocal stenoses with wall thickening/enhancement 11. [28] This strongly suggests that an immune response to A is responsible for CAA-RI. A is deposited segmentally, but can be found in all those inflammation sites. Typical images of cerebral amyloid angiopathy-related inflammation. modify the keyword list to augment your search. [55,56] Thus, 2 carriers may also be predisposed to CAA-RI. 32. Third, A was engulfed by macrophages expressing MHC class II antigens near CD4+ T cells, suggesting that A plays a pathogenic role in inducing inflammation in ABRA. Impact of A40 and A42 Fibrils on the Transcriptome of Primary Astrocytes and Microglia. Association between immunosuppressive treatment and outcomes of cerebral amyloid angiopathy-related inflammation. Clinical history of progressive cognitive decline over a few weeks and asymmetrically grouped cerebral microbleeds with focal corticosubcortical FLAIR hyperintensity, untypical for stroke and without restricted diffusion, we suspected cerebral amyloid angiopathy related inflammation (CAA-RI). The aim of future research should focus on specific pathogenic mechanisms and inflammatory pathways to determine which types of CAA patients are prone to developing inflammation, whether other genes or alleles besides APOE 4 are also risk factors, how they play a role in the mechanism, and so on. Epub 2022 Mar 14. may email you for journal alerts and information, but is committed Due to the potentially reversible WMH in ICAA,[43] when clinical manifestations are present and findings on conventional MRI sequences are suggestive, it must be distinguished from PRES, which also has the characteristic of bilateral confluent T2 WMH, but is often associated with hypertension or other conditions. Nelson T, Leung B, Bannykh S, Shah KS, Patel J, Dumitrascu OM. [68] Other features include seizures, headaches, T2-weighted white matter hyperintense (WMH) lesions on magnetic resonance imaging (MRI), and pathological evidence of inflammation against vascular A, which is the hallmark of CAA. Cerebral amyloid angiopathy-related inflammation (CAAri) is characterized by vasogenic edema and multiple cortical/subcortical microbleeds, sharing several aspects with the recently defined amyloidrelated imaging abnormalities (ARIA) reported in Alzheimer's disease (AD) passive immunization therapies. [22] Moreover, ischemic stroke is more common in PACNS than in CAA-RI,[24] and there have been only a few cases of patients with CAA-RI presenting with ischemic stroke.