Nakaya M, Hashimoto H, Usui G, Sawada K, Shirouzu I, Oshima A, Okubo S, Yamada H, Morikawa T. Cardiovasc Pathol. (2016) Journal of Alzheimer's disease : JAD. There are two recognized pathologically characterized variants: cerebral amyloid angiopathy-related inflammation (CAAri) and A beta-related angiitis (ABRA). Magnetic resonance angiography (MRA) or cerebral angiography is unremarkable in CAA-RI, due to the small caliber of the involved blood vessels, which prevents the lesion from being captured. 17. [46] Two-thirds of ABRA patients and only 31.3% of ICAA patients showed contrast enhancement on MRI. Reference article, Radiopaedia.org (Accessed on 01 Mar 2023) https://doi.org/10.53347/rID-28025, Posterior reversible encephalopathy syndrome (PRES) with intracerebral, intraventricular hemorrhage and cerebral vasculopathy, Amyloid-related imaging abnormalities (ARIA), amyloid-related imaging abnormalities (ARIA), progressive multifocal leukoencephalopathy (PML), posterior reversible encephalopathy syndrome (PRES), Cerebral amyloid inflammatory vasculopathy, Cerebral amyloid angiopathy related inflammation (CAA-ri), Cerebral amyloid angiopathy associated with giant cell arteritis. [14,29] Finally, in terms of clinical manifestations and prognosis, there was no difference between the two pathological subtypes of CAA-RI. Miller-Thomas MM, Sipe AL, Benzinger TL et-al. 2015 Sep;24(9):e245-50. 39. A report of 2 cases. Biomedicines. Nat Rev Neurol. Martucci M, Sarria S, Toledo M et-al. 57. Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare but increasingly recognized subtype of CAA. [40] Whether the etiology of these comorbidities, such as autoimmunity, or their treatment, such as radiation therapy,[41] are related to CAA-RI requires further study. Cerebral amyloid angiopathy with related inflammation (CAA-RI) is an uncommon inflammatory subtype of CAA, with a variety of presentations that can mimic other focal and diffuse neurological disorders. A clinico-radiological study of cerebral amyloid angiopathy-related inflammation. [22,31] In fact, both ICAA and ABRA can present with or without granulomatous inflammation. 8. [39] One patient with a history of Parkinson's disease (PD) was mistakenly thought to have developed the mental manifestation of PD when he presented with the symptoms of CAA-RI. Curr Neurol Neurosci Rep. 2015 Aug;15(8):54. doi: 10.1007/s11910-015-0572-y. [11] The most commonly used immunosuppressants are cyclophosphamide (33.9%), azathioprine (5.0%), mycophenolate mofetil (5.0%), methotrexate, immunoglobulin, and so on. 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. Keyword Highlighting This also reflects the importance of the SWI sequence. Cerebral amyloid--related angiitis without cerebral microbleeds in a patient with subarachnoid hemorrhage. 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. [61] Despite this, negative brain biopsy findings are insufficient to exclude the diagnosis of CAA-RI, because of the segmental distribution of pathological changes. Carmona-Iragui M, Fernndez-Arcos A, Alcolea D, Piazza F, Morenas-Rodriguez E, Antn-Aguirre S, et al. Acute or subacute onset of cognitive decline or behavioral changes is the mos J Alzheimers Dis. Typical images of cerebral amyloid angiopathy-related inflammation. Our clinical experience also supports this conclusion [Figure 1]. Amyloid--related angiitis: a report of 2 cases with unusual presentations. Check for errors and try again. However, biopsy is invasive; consequently, some criteria for the diagnosis of CAA-RI have been based on clinical and radiological data. doi: 10.5853/jos.2015.17.1.17. The rare forms of inflammatory angiopathy attributed to A, A-related angiitis . 6. 7. Cerebral amyloid angiopathy and cerebral amyloid angiopathy-related inflammation: comparison of hemorrhagic and DWI MRI features. (A) Confluent WMH. Anti-amyloid beta autoantibodies in cerebral amyloid angiopathy-related inflammation: implications for amyloid-modifying therapies. Epub 2019 May 25. Department of Neurology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China. It may also be possible that, due to sampling error on biopsy,the pathological diagnosis does not reflect the global picture depicted on imaging 6. However, clinicoradiological diagnostic criteria have been proposed for cerebral amyloid angiopathy related inflammation 4. at least one of these clinical features not directly attributable to an acute intracerebral hemorrhage: MRI with white matter hyperintensities (unifocal or multifocal, corticosubcortical or deep) that extend to the immediately subcortical white matter, at least one of these corticosubcortical hemorrhagic lesions: cerebral macrobleed, cerebral microbleed, or cortical superficial siderosis, absence of neoplastic, infectious, or other cause. Probatory corticoid treatment resolved FLAIR changes . CMBs: Cerebral microbleeds; WMH: White matter hyperintensity. Still others refer to only cerebral amyloid angiopathy-related inflammation alone 1,4,5,10or amyloid -related angiitis alone 7without mention of the other. Leptomeningeal enhancement may be a unique imaging manifestation in some cases with confirmed CAA-RI. 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. Kirshner et al[8] reported a CAA-RI patient with pathologically confirmed grade III anaplastic astrocytoma. 5. [4] With the development of imaging technology, more clinical silent patients are identified by the classic imaging abnormalities, including multiple strictly lobar cerebral microbleeds (CMBs), cortical superficial siderosis (cSS) or cortical subarachnoid hemorrhage, and cortical atrophy.[3]. [54] Therefore, the presence of the APOE 4/4 genotype may be meaningful for the diagnosis of CAA-RI. 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. In particular, amiloid tracers revealed higher retention in CAA patients, correlation with cerebral bleed, the ability to differentiate between CAA and other related conditions (such as Alzheimer's disease) and a correlation with some cerebrospinal fluid biomarkers. Amyloid PET is also unavailable in most hospitals in China. Pathogenetical subtypes of recurrent intracerebral hemorrhage: designations by SMASH-U classification system. Cerebral amyloid angiopathy-related inflammation (CAA-ri) is an unusual cause of encephalopathy, seizures and focal neurological deficits.1 2 We report three cases of CAA-ri with minimal symptoms but striking and dynamically evolving brain MRI findings. (2016) Neurology. 11C-PiB PET imaging of encephalopathy associated with cerebral amyloid angiopathy. Amyloid-related imaging abnormalities in patients with Alzheimer's disease treated with bapineuzumab: a retrospective analysis. Ronsin S, Deiana G, Geraldo AF, Durand-Dubief F, Thomas-Maisonneuve L, Formaglio M, et al. [14] Previous studies have revealed that, compared with multiple sclerosis and healthy people, anti-A autoantibodies in the CSF of CAA-RI patients increased during the acute phase, which is consistent with what was observed in ARIA, supporting the aforementioned hypothesis of an A-induced immune response. There are also cases of CAA-RI patients reported with genotype APOE 2/2 and APOE 2/3. Would you like email updates of new search results? Vonsattel grading for CAA severity on neuropathology samples. 2016YFC1300500-505). 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. [13] For patients diagnosed with probable CAA-RI by means of these criteria, immunosuppressive therapy can be given empirically to avoid brain biopsy. Moosavi B, Torres C, Jansen G. Case 232: amyloid--related angiitis. Traschtz A, Tzaridis T, Penner AH, Kuchelmeister K, Urbach H, Hattingen E, et al. 52. Savoiardo M, Erbetta A, Storchi G, Girotti F. Case 159: cerebral amyloid angiopathy-related inflammation. Although CAA-RI is relatively rare at present, it may become more common in future with the improvement of diagnostic techniques. 67. Cerebral amyloid angiopathy (CAA) is a type of cerebrovascular disorder characterized by the accumulation of amyloid within the leptomeninges and small/medium-sized cerebral blood vessels. 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. Wolters Kluwer Health Primary central nervous system vasculitis: comparison of patients with and without cerebral amyloid angiopathy. Brashear, H.M. Arrighi, K.A. your express consent. Impact of A40 and A42 Fibrils on the Transcriptome of Primary Astrocytes and Microglia. The gold standard for diagnosis is autopsy or brain biopsy. Chin Med J 2021;134:646654. The most recent systematic review included 213 pathologically confirmed cases of CAA-RI. Cerebral amyloid angiopathy is a common small vessel disease in the elderly involving vascular amyloid- deposition. There are two recognized pathologically characterized variants: cerebral amyloid angiopathy-related inflammation (CAAri) and A beta-related angiitis (ABRA). [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. 50. Salvarani C, Morris JM, Giannini C, Brown RD Jr, Christianson T, Hunder GG. (C) No enhancement was seen. 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. Morris, M. Grundman. [2,1719] In addition, some researchers still believe that CAA-RI/ICAA and ABRA are two different disease entities. Cerebral amyloid angiopathy-related inflammation: imaging findings and clinical outcome. Amyloid can be confirmed when the Congo red-stained section shows green birefringence under polarized light. The distribution of CMBs does not follow the regional pattern of occipital dominance in non-inflammatory CAA. Unable to load your collection due to an error, Unable to load your delegates due to an error. In autopsy series, the estimated prevalence of CAA is high (20-40 % in nondemented subjects; 50-60 % in dementia) [1]. [50,51] In these extreme cases, brain biopsy seems to be the only choice. This study was supported by a grant from the National Key Research and Development Program of China (No. In addition, when starting the treatment, infection needs to be ruled out first, to avoid pervasion due to corticosteroid therapy. Cerebral amyloid angiopathy-related inflammation: a case report presenting with a rare variant in SORL1 gene. [14] However, findings from another study have suggested that non-specific vascular changes in ABRA may be observed when medium-sized arteries are involved. Cerebral amyloid angiopathy-related inflammation. Renard D, Tatu L, Collombier L, Wacongne A, Ayrignac X, Charif M, et al. It may present with symptomatic acute lobar intracerebral hemorrhage (ICH), chronic progressive cognitive decline, transient focal neurological episodes, and subacute cognitive disorder or behavioral changes caused by CAA-related inflammation (CAA-RI). Kinnecom C, Lev MH, Wendell L, Smith EE, Rosand J, Frosch MP, et al. 51 (2): 525-32. 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. 37. Update of hot topics in neuralogic diseases. 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. Brain Nerve. Other diagnostic indexes include the apolipoprotein E 4 allele, A and anti-A antibodies in cerebral spinal fluid and amyloid positron emission tomography. (2016) Medicine. Raghavan P, Looby S, Bourne TD, Wintermark M. Cerebral amyloid angiopathy-related inflammation: a potentially reversible cause of dementia with characteristic imaging findings. [64] Another patient was first diagnosed with PRES, which was responsive to anti-edema intravenous steroid and antihypertensive therapy. 31. Clipboard, Search History, and several other advanced features are temporarily unavailable. DiFrancesco JC, Brioschi M, Brighina L, Ruffmann C, Saracchi E, Costantino G, et al. 5. An increase in inflammatory biomarkers has been observed in CAA-RI patients in different studies. There have been few epidemiological studies on CAA-RI. 72. 2018;64(4):1113-1121. doi: 10.3233/JAD-180269. 35. [9,10] Two pathological subtypes are now generally accepted: non-destructive perivascular inflammation (inflammatory CAA [ICAA]) and transmural or intramural inflammation (A-related angiitis [ABRA]). 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. [17] And the youngest case with pathologic evidence ever reported was 42 years old.[34]. 95 (20): e3613. Brain Pathol. 8. 280 (2): 643-7. [14] Based on the criteria of leptomeningeal enhancement, the sensitivity and specificity of recognizing CAA-RI from CAA patients are reported to be 70.4% and 92.6%, respectively. [18] However, these results should be carefully considered because the high proportion of granulomatous inflammation may be due to the higher biopsy rate in those cases showing more serious clinical and imaging manifestations and a tendency of malignant diseases.[22]. Validation of Clinicoradiological Criteria for the Diagnosis of Cerebral Amyloid Angiopathy-Related Inflammation. Tetsuka S, Hashimoto R. Slightly symptomatic cerebral amyloid angiopathy-related inflammation with spontaneous remission in four months. Multimodality Review of Amyloid-related Diseases of the Central Nervous System. doi: 10.1097/CM9.0000000000001427, This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited.