[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. 6. The diagnosis of inflammatory cerebral amyloid angiopathy on clinicoradiologic grounds requires the exclusion of other causes: amyloid-related imaging abnormalities (ARIA)seen in patients treated with amyloid lowering therapies 13, infection, such as progressive multifocal leukoencephalopathy (PML)or meningoencephalitis of various causes, vascular pathologies, such as primary CNS vasculitisor posterior reversible encephalopathy syndrome (PRES), Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. 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]. The diagnostic criteria for possible or probable inflammatory cerebral amyloid angiopathy require at least one of the following clinical features that are not directly attributable to an acute intracerebral hemorrhage4: Some patients also present with hallucinations 2. Imaging Findings of Cerebral Amyloid Angiopathy, A-Related Angiitis (ABRA), and Cerebral Amyloid Angiopathy-Related Inflammation: A Single-Institution 25-Year Experience. Anti-A autoantibodies in the CSF of a patient with CAA-related inflammation: a case report. [2] CAA is clinically diverse. 36. Bookshelf [18] Although the APOE 2 allele is considered a protective factor against AD, it clearly increases the risk of vascular disease. 45. [2023] In recent years, it has gradually come to be accepted that these two pathological types are essentially similar. [12,14,18] The erythrocyte sedimentation rate was increased in 37.5% of patients, while C-reactive protein (CRP) was elevated in 60%. Pathogenetical subtypes of recurrent intracerebral hemorrhage: designations by SMASH-U classification system. 6. 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. [61] Despite this, negative brain biopsy findings are insufficient to exclude the diagnosis of CAA-RI, because of the segmental distribution of pathological changes. Castro Caldas A, Silva C, Albuquerque L, Pimentel J, Silva V, Ferro JM. Cerebral amyloid angiopathy (CAA) is a condition in which proteins called amyloid build up on the walls of the arteries in the brain. In the remainder, which accounts for 60% of all affected individuals, even with treatment severe disability or death are encountered 2. 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. [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. Ronsin S, Deiana G, Geraldo AF, Durand-Dubief F, Thomas-Maisonneuve L, Formaglio M, et al. Morris, M. Grundman. There is currently no study giving recommendations on the choice of medication, dosage, and the time span of treatment. Acute or subacute onset of cognitive decline or behavioral changes is the most common symptom of CAA-RI. 2015 Sep;24(9):e245-50. 29. Due to these atypical symptoms, advanced imaging is very meaningful for clinical diagnosis. 71. Liang JW, Zhang W, Sarlin J, Boniece I. Salvarani C, Hunder GG, Morris JM, Brown RD, Christianson T, Giannini C. A-related angiitis: comparison with CAA without inflammation and primary CNS vasculitis. [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. 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). Unauthorized use of these marks is strictly prohibited. Acta Neuropathol. 31. A 77-year-old female experienced light-headedness during walking and mild ataxic gait without any other objective neuropsychological deficits. Moreover, amyloid deposits start in the cortical areas and spread to the hippocampal areas at a later stage [32,33]. 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. (C) No enhancement was seen. 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). In order to make a diagnosis before histopathology, Chung et al[12] proposed the Boston criteria using clinicoradiological data in 2011. Disclaimer. An official website of the United States government. Inflammatory Cerebral Amyloid Angiopathy, Amyloid-Related Angiitis, and Primary Angiitis of the Central Nervous System. Neurology 2013; 81:15961603. [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. The case of an 85-year-old female with acute right hemiparesis with status epilepticus. 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. [3] CAA related lobar ICH has been identified as the second most common form of spontaneous ICH following hypertensive angiopathy. Moreover, ABRA was considered to be different from ICAA because it has the same vascular destructive pathological changes as PACNS. 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. Careers. [17] While another systematic review showed that the functional outcome of most patients was not ideal. Medicine (Baltimore). Some authors are consistent with the terms we have used here, while some call the two subtypes CAA-RI and ABRA. Kang P, Bucelli RC, Ferguson CJ, Corbo JC, Kim AH, Day GS. CMBs: Cerebral microbleeds; WMH: White matter hyperintensity. Epub 2022 Aug 5. SWI or T2: which MRI sequence to use in the detection of cerebral microbleeds? Biopsy obtained from the white matter showed no evidence of inflammation in one case. This site needs JavaScript to work properly. 30. 57. your express consent. 32. Imaging findings of cerebral amyloid angiopathy, Abeta-related angiitis (ABRA), and cerebral amyloid angiopathy-related inflammation: a single-institution 25-year experience. 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. Before Du Y, Liu C, Ma C, Xu X, Zhou X, Zhou H, et al. Cerebral amyloid angiopathy (CAA) is characterized by amyloid beta-peptide deposits within small- to medium-sized blood vessels of the brain and leptomeninges. Hemorrhage and white matter injury seen at imaging reflect vascular damage caused by the accumulation of A in vessel walls. In the vast majority of cases (90%), microhemorrhages are present 1,2. After treatment with corticoids, (D) WMH faded significantly. However, anticoagulation was later suspended due to cerebral hemorrhage, and the patient was finally diagnosed with CAA-RI. [18] No difference in outcome was found between patients receiving mono-therapy of corticosteroid and patients receiving a combination of immunosuppressant and corticosteroid therapy. Cerebral amyloid angiopathy (CAA) is an important cause of cognitive impairment and spontaneous intracerebral hemorrhage in the elderly. [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. Nakaya M, Hashimoto H, Usui G, Sawada K, Shirouzu I, Oshima A, Okubo S, Yamada H, Morikawa T. Cardiovasc Pathol. (2016) JAMA neurology. Another option is to follow the patient up closely. Corovic A, Kelly S, Markus HS. J Stroke 2015; 17:1730. CD4(+) T cells predominate in cerebrospinal fluid and leptomeningeal and parenchymal infiltrates in cerebral amyloid beta-related angiitis. Sallles E, Bonneville F, Delisle MB, Rigal E, Raposo N, Pariente J. modify the keyword list to augment your search. One case was initially suspected of PRES or cerebral venous sinus thrombosis and was treated with anticoagulant and steroid. If the brain biopsy result is negative, but the patient meets the clinicoradiological diagnostic criteria, the course of action remains uncertain. Ichimata S, Hata Y, Yoshida K, Nishida N. Autopsy of a multiple lobar hemorrhage case with amyloid--related angiitis. Phrases such as CAA associated with inflammation, CAA-RI, ICAA, and ABRA are used interchangeably. Nationwide survey on cerebral amyloid angiopathy in Japan. An increase in inflammatory biomarkers has been observed in CAA-RI patients in different studies. Search for Similar Articles doi: 10.1111/bpa.13061. 15 (8): 54. -. Recurrence of cerebral amyloid angiopathy-related inflammation: a report of two cases from the iCAbeta international network. Moreover, the efficacy of treatment was evaluated by observational studies; consequently, more clinical trials and even randomized clinical trials are required. 2016;36 (4): 1147-63. Update of hot topics in neuralogic diseases. An intense perivascular inflammation with multinucleated giant cells is found in a minority of CAA patients, possibly those with an exaggerated inflammatory response to vascular leakages that occur from amyloid- laden arteries. Primary central nervous system vasculitis: comparison of patients with and without cerebral amyloid angiopathy. Renard D, Tatu L, Collombier L, Wacongne A, Ayrignac X, Charif M, et al. In addition, when starting the treatment, infection needs to be ruled out first, to avoid pervasion due to corticosteroid therapy. Many cases have reported that patients were misdiagnosed with tumors, and the diagnosis was modified to CAA-RI when the data were retrospectively analyzed or after the biopsy results became available. Perivascular and vascular inflammatory patterns without granulomas accounted for 22.5% of cases. The rare forms of inflammatory angiopathy attributed to A, A-related angiitis . Many studies have reported that APOE 4/4 homozygosity is significantly correlated with CAA-RI,[47] accounting for 76.9% of CAA-RI patients. 2022 Nov;43(11):6381-6387. doi: 10.1007/s10072-022-06299-y. Clipboard, Search History, and several other advanced features are temporarily unavailable. Xu YY, Chen S, Zhao JH, Chen XL, Zhang JW. 8. Conclusive diagnosis of CAA-RI requires histopathological confirmation, but it is invasive and has certain risks. Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Blood tests may reveal signs of inflammation. 2022 Jul;9(7):1102-1103. doi: 10.1002/acn3.51596. HHS Vulnerability Disclosure, Help Cerebral amyloid angiopathy is an increasingly important cause of hemorrhagic strokes in older adults, contributing to the growing vascular . Szpak GM, Lewandowska E, Sliwiska A, Stpie T, Tarka S, Mendel T, et al. A case of cerebral amyloid angiopathy-related inflammation with the rare apolipoprotein epsilon2/epsilon2 genotype. Cerebral amyloid angiopathy (CAA) is a cerebrovascular disorder caused by the accumulation of cerebral amyloid- (A) in the tunica media and adventitia of leptomeningeal and cortical vessels of the brain. 14. [50,51] In these extreme cases, brain biopsy seems to be the only choice. 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. Amyloid PET is also unavailable in most hospitals in China. 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. Other diagnostic indexes include the apolipoprotein E 4 allele, A and anti-A antibodies in cerebral spinal fluid and amyloid positron emission tomography. [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. Pseudotumoral presentation of cerebral amyloid angiopathy-related inflammation. . Cerebral amyloid angiopathy-related inflammation: imaging findings and clinical outcome. [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. Thus, PACNS is on the list of differential diagnoses whenever multifocal hyperintensity is seen on FLAIR images, although it is a diagnosis of exclusion. [55] An APOE 4/4 homozygous patient with a rare SORL1 mutation has been reported. An official website of the United States government. However, biopsy is invasive; consequently, most clinically diagnosed cases have been based on clinical and radiological data. 1 Introduction of the imaging-based Boston criteria for diagnosis of CAA in the 1990s 2, 3 Martucci M, Sarria S, Toledo M et-al. 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. It is conceivable that posterior reversible encephalopathy syndrome (PRES) is a very important differential diagnosis. Sperling R, Salloway S, Brooks DJ, Tampieri D, Barakos J, Fox NC, et al. 2018;64(4):1113-1121. doi: 10.3233/JAD-180269. 28. 59. 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. CAA-RI consists of two subtypes: inflammatory cerebral amyloid angiopathy and amyloid (A)-related angiitis. Thirteen percent of patients were affected with some forms of visual impairment. Beta-amyloid peptides bind to lipoproteins and apolipoproteins E and J in the CSF and to HDL particles in plasma, inhibiting metal-catalyzed oxidation of lipoproteins. Diagnosis, treatment, and follow-up of patients with cerebral amyloid angiopathy-related inflammation. (2015) Current neurology and neuroscience reports. Probable Cerebral Amyloid Angiopathy-Related Inflammation Associated With Sitravatinib: A Case Report. Cancelloni V, Rufa A, Battisti C, De Stefano N, Mastrocinque E, Garosi G, Venezia D, Chiarotti I, Cerase A. Neurol Sci. 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. Cerebral Amyloid Angiopathy-Related Inflammation: A Single-Center Experience and a Literature Review. 8. [1] The amyloid deposition results in fragile vessels that may manifest in brain bleeds. Hainline C, Rucker JC, Zagzag D, Golfinos JG, Lui YW, Liechty B, et al. 67. 2016;51(2):525-32. doi: 10.3233/JAD-151036. (B) Strictly lobar CMBs. Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare but increasingly recognized subtype of CAA. Unable to load your collection due to an error, Unable to load your delegates due to an error. 2019 Sep-Oct;42:36-40. doi: 10.1016/j.carpath.2019.05.004. 37. Boncoraglio GB, Piazza F, Savoiardo M, Farina L, DiFrancesco JC, Prioni S, et al. Carmona-Iragui M, Fernndez-Arcos A, Alcolea D, Piazza F, Morenas-Rodriguez E, Antn-Aguirre S, et al. Immune activation in amyloid--related angiitis correlates with decreased parenchymal amyloid- plaque load. [2527] ARIA is also divided into two categories: ARIA-E, which manifests as focal or confluent vasogenic edema on fluid-attenuated inversion recovery (FLAIR) sequence images, and ARIA-H, characterized by CMBs or cSS on T2-weighted gradient-echo/susceptibility-weighted imaging (SWI) sequence scans, corresponding to the image hallmarks of CAA-RI. Cerebral amyloid angiopathy-related inflammation in the immunosuppressed: a case report. 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. Thus, it needs to be established whether excessive immune suppression would have an adverse effect on the long-term prognosis of patients. Nat Rev Neurol. Risk factor SORL1: from genetic association to functional validation in Alzheimer's disease. PMC Probatory corticoid treatment resolved FLAIR changes . Yeh SJ, Tang SC, Tsai LK, Jeng JS. Federal government websites often end in .gov or .mil. CAA-RI is now widely recognized as a relatively rare and aggressive subtype of CAA with diverse clinical presentations and characteristic radiological findings. Tumefactive cerebral amyloid angiopathy mimicking CNS neoplasm. However, antibody titer determination kits are currently not commercially available and are still worth developing. About 60% of patients died or were severely disabled after immunotherapy, and there was no statistically significant difference in terms of prognosis between the two pathological types. Kirshner HS, Bradshaw M. The inflammatory form of cerebral amyloid angiopathy or cerebral amyloid angiopathy-related inflammation (CAARI). [14] The dosage used is based on individual selection. At present, the main recommendation is that high-dose glucocorticoids should be used.
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