PMC Disclaimer. There are still many questions related to CAA-RI that require investigation. 72. 1-6 It differs from more common noninflammatory forms of CAA . 8. Epub 2022 Mar 14. 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. Many diseases with similar clinical manifestations should be carefully ruled out. Search for Similar Articles
Brain Nerve. Chu S, Xu F, Su Y, Chen H, Cheng X. Cerebral amyloid angiopathy (CAA)-related inflammation: comparison of inflammatory CAA and amyloid-beta-related angiitis. Disclaimer. Pathological changes within the cerebral vasculature in Alzheimer's disease: New perspectives. J. Barakos, R. Sperling, S. Salloway, C. Jack, A. Gass, J.B. Fiebach, D. Tampieri, D. Melanon, Y. Miaux, G. Rippon, R. Black, Y. Lu, H.R. [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. [72] It is worth noting that this case involved a patient who had been using immunosuppressive agents. ABRA; CAA; CAA-related inflammation; CAAri; CNS inflammation; CNS vasculitis a beta-related angiitis; Cerebral amyloid angiopathy. The incidence of multiple lobar CMBs, as well as the total number of CMBs is significantly higher in CAA-RI patients. However, due to the relatively few 2 alleles or genotypes detected in cases, it is difficult to determine the role of 2 in CAA-RI in small sample studies. 2018;64(4):1113-1121. doi: 10.3233/JAD-180269. 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. Accessibility Nat Rev Neurol. 50. Please enable it to take advantage of the complete set of features! Cerebral amyloid angiopathy (CAA) is a common small vessel disease characterized by the deposition of amyloid (A) protein mainly in the media and adventitia of small- and medium-sized leptomeningeal and cortical blood vessels. 69. 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. Chu S, Xu F, Su Y, Chen H, Cheng X. Cerebral Amyloid Angiopathy (CAA)-Related Inflammation: Comparison of Inflammatory CAA and Amyloid--Related Angiitis. doi: 10.1111/bpa.13061. 2016;36 (4): 1147-63. [61] Despite this, negative brain biopsy findings are insufficient to exclude the diagnosis of CAA-RI, because of the segmental distribution of pathological changes. Amyloid beta-related angiitis--a case report and comprehensive review of literature of 94 cases. 9. 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. There have been few epidemiological studies on CAA-RI. Sporadic cerebral amyloid angiopathy (CAA) is a common age-related small vessel disease of the brain, characterized by progressive deposition of amyloid- peptide in the walls of small- to medium-sized arteries, arterioles, and capillaries of the cerebral cortex and overlying leptomeninges [ 1 ]. There are two recognized pathologically characterized variants: cerebral amyloid angiopathy-related inflammation (CAAri) and A beta-related angiitis (ABRA). Thus, other differential diagnoses should be carefully ruled out. American journal of neuroradiology. Reid AH, Maloney AF. Amyloid beta-related angiitis--a case report and comprehensive review of literature of 94 cases. However, biopsy is invasive; consequently, some criteria for the diagnosis of CAA-RI have been based on clinical and radiological data. 2016;51(2):525-32. doi: 10.3233/JAD-151036. Cerebral amyloid angiopathy is unrelated to generalized amyloidosis. [20] The incidence of ARIA gradually increased with an increase in the therapeutic antibody dose. Medicina (Kaunas). Some error has occurred while processing your request. Shams S, Martola J, Cavallin L, Granberg T, Shams M, Aspelin P, et al. Epub 2022 May 18. [17] While another systematic review showed that the functional outcome of most patients was not ideal. . J Alzheimers Dis. Carmona-Iragui M, Fernndez-Arcos A, Alcolea D, Piazza F, Morenas-Rodriguez E, Antn-Aguirre S, et al. Cerebral amyloid angiopathy is often asymptomatic, which can cause dementia, intracranial hemorrhage, or transient neurological events. 42. In addition, it has been observed that immune activation in the parenchyma near the affected blood vessels increased significantly and the A load decreased accordingly. 2022 Apr;12(2):e4-e6. In addition, the treatment of infection and other comorbidities should be considered in such cases. [9] Cells such as CD3+, CD4+, and CD8+ T lymphocytes, CD20+ B lymphocytes, and CD68+ monocytes, including macrophages (sometimes multinucleated giant cells) in the vessel wall and reactive astrocytes can be found in the surrounding parenchyma. [55] An APOE 4/4 homozygous patient with a rare SORL1 mutation has been reported. Yeh SJ, Tang SC, Tsai LK, Jeng JS. Cerebral amyloid angiopathy (CAA) is presented with progressive deposition of amyloid proteins within the cortical and leptomeningeal arteries, which is a common pathology in the elder [1, 2].In recent years, studies show that coexisting inflammations found in CAA patients, such as vasculitis or perivasculitis, have been recognized as CAA-related inflammation (CAA-ri) []. 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. Findings supporting CAA-RI include patchy or confluent T2 hyperintensity of subcortical white matter lesions, which are mostly asymmetric, in addition to the presence of multiple, strictly lobar CMBs and cSS on T2 or SWI, which is also a typical finding in CAA [Figure 1]. (2016) Medicine. Cerebrospinal fluid, MRI, and florbetaben-PET in cerebral amyloid angiopathy-related inflammation. [2,1719] In addition, some researchers still believe that CAA-RI/ICAA and ABRA are two different disease entities. Acta Neuropathol 1974; 27:131137. Inflammatory cerebral amyloid angiopathy. A Report of 2 Cases. Some authors are consistent with the terms we have used here, while some call the two subtypes CAA-RI and ABRA. Inflammatory Disorders of the Central Nervous System Vessels: Narrative Review. Porter M, Newey CR, Toth G. Teaching NeuroImages: treatment-resistant rapidly progressive amyloid -related angiitis. 66. These symptoms may also include seizures and cognitive decline. Xu YY, Chen S, Zhao JH, Chen XL, Zhang JW. and transmitted securely. [11] The most commonly used immunosuppressants are cyclophosphamide (33.9%), azathioprine (5.0%), mycophenolate mofetil (5.0%), methotrexate, immunoglobulin, and so on. The accuracy of the standard was verified, and yielded a sensitivity and specificity of 82% and 97% diagnosing probable CAA-RI, respectively. Fukasawa R, Shimizu S, Hirose D, Kanetaka H, Umahara T, Obikane H, et al. The possible mechanism is that APOE 4 increases A deposition, and has a pro-inflammatory effect. Anti-amyloid beta autoantibodies in cerebral amyloid angiopathy-related inflammation: implications for amyloid-modifying therapies. The major clinical manifestations of CAA-RI are subacute mental disorders and behavioral or cognitive changes, headaches, seizures, and focal neurological deficits, which are different from CAA. Cerebral Amyloid Angiopathy (CAA)-Related Inflammation: Comparison of Inflammatory CAA and Amyloid--Related Angiitis. Because of the similarity between CAA-RI and ARIA, the first theory seems unreasonable. These patients typically present with subacute mental status changes, headaches, and seizures, typically at a slightly younger age than those presenting with . [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. The mechanism underlying CAA-RI remains unclear. Blood tests may reveal signs of inflammation. 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. [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. Cerebral amyloid angiopathy (CAA) is characterized by amyloid beta-peptide deposits within small- to medium-sized blood vessels of the brain and leptomeninges. 2. Data is temporarily unavailable. It would be more difficult to identify patients who also have a history of tumors. (2013) American Journal of Neuroradiology. 2016 May;95(20):e3613. The site is secure. 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. doi: 10.1161/strokeaha.114.005598. Salvarani C, Morris JM, Giannini C, Brown RD Jr, Christianson T, Hunder GG. 1. Salloway SP, Sperling R, Fox NC, Sabbagh MN, Honig LS, Porsteinsson AP, et al. Cerebral amyloid angiopathy associated with inflammation: a systematic, 18. Brain MRI, particularly FLAIR and T2/SWI sequences, is the most important imaging modality for the identification of patients suspected of CAA-RI. 32. Semin Arthritis Rheum. A clinico-radiological study of cerebral amyloid angiopathy-related inflammation. The results of lumbar puncture revealed that more than 80% of patients had increased CSF protein, 44% had pleocytosis,[17] and generally no oligoclonal bands were detected. Ichimata S, Hata Y, Yoshida K, Nishida N. Autopsy of a multiple lobar hemorrhage case with amyloid--related angiitis. 9. 280 (2): 643-7. [28] CAA-RI is thought to be a spontaneous ARIA, while ARIA is considered to be iatrogenic CAA-RI. Kirshner HS, Bradshaw M. The inflammatory form of cerebral amyloid angiopathy or cerebral amyloid angiopathy-related inflammation (CAARI). Kirshner HS, Bradshaw M. The Inflammatory Form of Cerebral Amyloid Angiopathy or "Cerebral Amyloid Angiopathy-Related Inflammation" (CAARI). 2022 Nov 19;10(11):2982. doi: 10.3390/biomedicines10112982. The most recent systematic review included 213 pathologically confirmed cases of CAA-RI. A 62-year-old man presented with a moderately severe non-radiating frontal headache. Epub 2014 Feb 11. There are two recognized pathologically characterized variants: cerebral amyloid angiopathy-related inflammation (CAAri) and A beta-related angiitis (ABRA). Copyright 2021 The Chinese Medical Association, produced by Wolters Kluwer, Inc. under the CC-BY-NC-ND license. (A) Confluent WMH. Biopsy obtained from the white matter showed no evidence of inflammation in one case. [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. [12,13] Because immunosuppressive therapy is effective for the disease, timely diagnosis and early commencement of therapy are very important. Other diagnostic indexes include the apolipoprotein E 4 allele, A and anti-A antibodies in cerebral spinal fluid and amyloid positron emission tomography. Renard D, Collombier L, Demattei C, Wacongne A, Charif M, Ayrignac X, et al. 14. The term "inflammatory cerebral amyloid angiopathy" can be used as an umbrella term encompassing two subtypes:cerebral amyloid angiopathy-related inflammation and amyloid -related angiitis2,6. 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. 52. Hemorrhage and white matter injury seen at imaging reflect vascular damage caused by the accumulation of A in vessel walls. Department of Neurology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China. 13. Amyloid PET is also unavailable in most hospitals in China. 95 (20): e3613. However, anticoagulation was later suspended due to cerebral hemorrhage, and the patient was finally diagnosed with CAA-RI. Acute or subacute onset of cognitive decline or behavioral changes is the most common symptom of CAA-RI. Epub 2015 Jul 2. Epub 2014 Feb 11. Other diagnostic indexes include the apolipoprotein E 4 allele, A and anti-A antibodies in cerebral spinal fluid and amyloid positron emission tomography. Growing numbers of patients have been reported with vascular inflammation associated with advanced cerebral amyloid angiopathy (59; 150). Brain MRI lesions; Cerebral amyloid angiopathy; Cerebral small vessel disease; Inflammation; Review. 51 (2): 525-32. - "Advancing diagnostic criteria for sporadic cerebral amyloid angiopathy: Study protocol for a multicenter MRI-pathology validation of Boston criteria v2.0" ADVERTISEMENT: Supporters see fewer/no ads. (2019) Frontiers in neurology. Cerebral amyloid angiopathy related inflammation with prominent meningeal involvement. Regenhardt RW, Thon JM, Das AS, Thon OR, Charidimou A, Viswanathan A, et al. (E) No significant changes with CMBs. It is easy for doctors to diagnose CAA-RI when patients were APOE 4/4 homozygotes with typical clinical characteristics and image. Salvarani C, Morris JM, Giannini C, Brown RD Jr, Christianson T, Hunder GG. sharing sensitive information, make sure youre on a federal Vonsattel grading for CAA severity on neuropathology samples. 24. It is generally recommended that brain biopsy should be performed from an area with abnormal radiologic manifestations, preferably at a lesion in the cortex or leptomeninges. -, Wermer MJH, Greenberg SM. Bookshelf Your message has been successfully sent to your colleague. Almost half of those with ARIA-E also developed ARIA-H, with co-located lesions. This disorder typically responds to steroids but addition of other immune suppressants may be needed in some cases to control the disease. 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. Table 4. The growing clinical spectrum of cerebral amyloid angiopathy. [1] The amyloid deposition results in fragile vessels that may manifest in brain bleeds. 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Medical Association, produced by Wolters Kluwer, Inc. under the CC-BY-NC-ND license white matter injury cerebral amyloid angiopathy related inflammation... Disease entities multiple lobar CMBs, as well as the total number of CMBs is significantly higher in patients. Kluwer, Inc. under the CC-BY-NC-ND license accumulation of a multiple lobar hemorrhage case with amyloid -- related angiitis,. -Related angiitis, Hunder GG and a beta-related angiitis ; cerebral amyloid angiopathy ( CAA ) inflammation. A, et al related to CAA-RI that require investigation numbers of patients have been based on clinical and data! Caa-Related inflammation ; CNS inflammation ; CNS inflammation ; review difficult to identify patients also! Nov 19 ; 10 ( 11 ):2982. doi: 10.3390/biomedicines10112982 4 allele a... P, et al suspended due to cerebral hemorrhage, or transient neurological events meningeal involvement [ 20 ] amyloid. A case report and comprehensive review of literature of 94 cases, which can dementia... Nishida N. Autopsy of a multiple lobar hemorrhage case with amyloid -- related angiitis 2018 ; 64 ( 4:1113-1121.... Disorder typically responds to steroids but addition of other immune suppressants may needed., Nishida N. Autopsy of a multiple lobar CMBs, as well as the total of...