[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. The .gov means its official. Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare but increasingly recognized subtype of CAA. 58. One case was initially suspected of PRES or cerebral venous sinus thrombosis and was treated with anticoagulant and steroid. 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. WMHs sometimes extend to the cortex with a mass effect showing hyperintensity in maps of apparent diffusion coefficient suggesting vasogenic edema. [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. 2014 Aug;44(1):86-92. doi: 10.1016/j.semarthrit.2014.02.001. If only routine sequences are performed, it is easy to mistake WMH as the only image manifestation and consequently delay diagnosis and treatment. 7. 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. Epub 2019 May 25. ADVERTISEMENT: Supporters see fewer/no ads. (C) No enhancement was seen. (2019) Frontiers in neurology. 6. Reid and Maloney first described CAA with vascular inflammation in a patient with AD in 1974, and subsequent cases were reported. Masrori P, Montagna M, De Smet E, Loos C. Posterior reversible encephalopathy syndrome caused by cerebral amyloid angiopathy-related inflammation. Curr Neurol Neurosci Rep. 2015 Aug;15(8):54. doi: 10.1007/s11910-015-0572-y. Thus, other differential diagnoses should be carefully ruled out. Pathological changes within the cerebral vasculature in Alzheimer's disease: New perspectives. CT and MRI demonstrate an area of vasogenic edema involving the subcortical white matter 1. The gold standard for diagnosis is autopsy or brain biopsy. Salloway SP, Sperling R, Fox NC, Sabbagh MN, Honig LS, Porsteinsson AP, et al. Acute or subacute onset of cognitive decline or behavioral changes is the most common symptom of CAA-RI. 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. [46] Two-thirds of ABRA patients and only 31.3% of ICAA patients showed contrast enhancement on MRI. Ann Neurol 2013; 73:449. Nationwide survey on cerebral amyloid angiopathy in Japan. 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. Ann Clin Transl Neurol. [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. -, Reid AH, Maloney AF. An official website of the United States government. [19] Spontaneous remission has been reported in some cases,[7,71] the fundamentals of which are not yet known. Cerebral amyloid angiopathy related inflammation (CAA-ri) is a rare encephalopathy resulting from perivascular inflammation after -amyloid (A) deposition in cerebral vessels leading to progressive dementia, focal neurological signs, seizures and intracerebral hemorrhages. (E) No significant changes with CMBs. 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. 3. [1] The amyloid deposition results in fragile vessels that may manifest in brain bleeds. Unable to load your collection due to an error, Unable to load your delegates due to an error. 256 (1): 323-7. Amyloid-related imaging abnormalities in patients with Alzheimer's disease treated with bapineuzumab: a retrospective analysis. Some authors are consistent with the terms we have used here, while some call the two subtypes CAA-RI and ABRA. Please try again soon. There are also cases of CAA-RI patients reported with genotype APOE 2/2 and APOE 2/3. The mechanism underlying CAA-RI remains unclear. CMBs: Cerebral microbleeds; WMH: White matter hyperintensity. Although CAA-RI is relatively rare at present, it may become more common in future with the improvement of diagnostic techniques. 27. Chinese Medical Journal134(6):646-654, March 20, 2021. 73 (2): 197-202. 33. Cerebral Amyloid Angiopathy-Related Inflammation: A Single-Center Experience and a Literature Review. Case of cerebral amyloid angiopathy-related inflammation - is the absence of cerebral microbleeds a good prognostic sign? 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. Cerebral amyloid angiopathy-related inflammation: imaging findings and clinical outcome. Yamada M. Cerebral amyloid angiopathy: emerging concepts. 14. [18] No difference in outcome was found between patients receiving mono-therapy of corticosteroid and patients receiving a combination of immunosuppressant and corticosteroid therapy. [72] It is worth noting that this case involved a patient who had been using immunosuppressive agents. Lesions are usually unifocal but multifocal involvement is occasionally present at the time of diagnosis (~30%)1. Immune activation in amyloid--related angiitis correlates with decreased parenchymal amyloid- plaque load. 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. Update of hot topics in neuralogic diseases. Castro Caldas A, Silva C, Albuquerque L, Pimentel J, Silva V, Ferro JM. Boncoraglio GB, Piazza F, Savoiardo M, Farina L, DiFrancesco JC, Prioni S, et al. An alternative transcript of the Alzheimer's disease risk gene SORL1 encodes a truncated receptor. An increase in inflammatory biomarkers has been observed in CAA-RI patients in different studies. Prodromal Alzheimer's disease presenting as cerebral amyloid angiopathy-related inflammation with spontaneous amyloid-related imaging abnormalities and high cerebrospinal fluid anti-A autoantibodies. [57]SORL1 encodes a 250-kDa protein called sorting protein-related receptor with A-type repeats (SorLA), which reduces the production and deposition of A peptides by regulating the processing of APP. 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). Acute or subacute onset of cognitive decline or behavioral changes is the most common symptom of CAA-RI. 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. Keywords: Our clinical experience also supports this conclusion [Figure 1]. Thomas Tropea, Prasad Shirvalkar, Krithiga Sekar, Kyung-Wha Kim, Apostolos Tsiouris, Ehud Lavi, Alan Segal. 67. 30. doi: 10.1111/bpa.13061. [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. Teaching neuro: cerebral amyloid angiopathy-related inflammation presenting with isolated leptomeningitis. Hao Q, Tsankova NM, Shoirah H, Kellner CP, Nael K. Vessel Wall MRI Enhancement in Noninflammatory Cerebral Amyloid Angiopathy. Many diseases with similar clinical manifestations should be carefully ruled out. Reduction of microbleeds by immunosuppression in a patient with A-related vascular inflammation. [58,59] Thus, a variant in SORL1 may lead to dysfunction of SorLA, eventually adding to the risk of CAA-RI. Salvarani C, Morris JM, Giannini C, Brown RD Jr, Christianson T, Hunder GG. 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. Corovic A, Kelly S, Markus HS. Brashear, H.M. Arrighi, K.A. 11. A nationwide survey demonstrated that its prevalence is about 0.13 per 100,000 population in Japan. A report of 2 cases. [18] Sakai et al[32] reported a case of CAA-RI at the chronic stage, with persistently elevated proteinase 3-antineutrophil cytoplasmic antibody levels. 66. A 62-year-old man presented with a moderately severe non-radiating frontal headache. A significant proportion of patients respond readily to treatment with corticosteroids, with or without a cytostatic agent,with improvement evident within a week or two of commencement of treatment. Carmona-Iragui M, Fernndez-Arcos A, Alcolea D, Piazza F, Morenas-Rodriguez E, Antn-Aguirre S, et al. 54. It is easy for doctors to diagnose CAA-RI when patients were APOE 4/4 homozygotes with typical clinical characteristics and image. 2022 Nov 19;10(11):2982. doi: 10.3390/biomedicines10112982. A 77-year-old female experienced light-headedness during walking and mild ataxic gait without any other objective neuropsychological deficits. Kang P, Bucelli RC, Ferguson CJ, Corbo JC, Kim AH, Day GS. [22] The mainstream view is that granulomatous inflammation is the pathological hallmark of ABRA, but not of ICAA. 2022 Nov;43(11):6381-6387. doi: 10.1007/s10072-022-06299-y. DiFrancesco JC, Touat M, Caulo M, Gallucci M, Garcin B, Levy R, et al. 3. [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. 2022 Jul;9(7):1102-1103. doi: 10.1002/acn3.51596. 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). An individual with cerebral amyloid angiopathy-related inflammation who displayed involuntary movements. WMH and vasogenic edema accompanied by a mass effect make brain tumors a highly suspected differentiation. [46,47] A possible explanation for this finding is that, once an immune response to vascular amyloid protein is generated, it affects multiple regions of brain via the spread of antibodies. Renard D, Wacongne A, Ayrignac X, Charif M, Fourcade G, Azakri S, et al. However, antibody titer determination kits are currently not commercially available and are still worth developing. An official website of the United States government. The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). [70] The clinical features of relapse are widely distributed, among which the decline of cognitive function and encephalopathy are the most common symptoms. 10. [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. 22. (2020) AJNR. CAA-RI consists of two subtypes: inflammatory cerebral amyloid angiopathy and amyloid (A)-related angiitis. Discussion This report of neurologic autoimmunity in a patient receiving sitravatinib opens new lines of inquiry into the pathophysiology of CAA-ri. [2,1719] In addition, some researchers still believe that CAA-RI/ICAA and ABRA are two different disease entities. Cerebral amyloid angiopathy (CAA) is a condition in which proteins called amyloid build up on the walls of the arteries in the brain. Cancelloni V, Rufa A, Battisti C, De Stefano N, Mastrocinque E, Garosi G, Venezia D, Chiarotti I, Cerase A. Neurol Sci. government site. Other diagnostic indexes include the apolipoprotein E 4 allele, A and anti-A antibodies in cerebral spinal fluid and amyloid positron emission tomography. Cerebral amyloid angiopathy associated with inflammation: report of 3 cases and systematic. Auriel E, Charidimou A, Gurol ME, Ni J, Van Etten ES, Martinez-Ramirez S, et al. to maintaining your privacy and will not share your personal information without
Inflammatory cerebral amyloid angiopathy: the overlap of perivascular (PAN-like) with vasculitic (A-related angiitis) form: an autopsy case. Thus, PACNS is on the list of differential diagnoses whenever multifocal hyperintensity is seen on FLAIR images, although it is a diagnosis of exclusion. It is worth noting that CAA-RI is a diagnosis by exclusion. Imaging Findings of Cerebral Amyloid Angiopathy, A-Related Angiitis (ABRA), and Cerebral Amyloid Angiopathy-Related Inflammation: A Single-Institution 25-Year Experience. Tumoral presentation of homonymous hemianopia and prosopagnosia in cerebral amyloid angiopathy-related inflammation. A Report of 2 Cases. The accuracy of the standard was verified, and yielded a sensitivity and specificity of 82% and 97% diagnosing probable CAA-RI, respectively. . Liang JW, Zhang W, Sarlin J, Boniece I. 19. Nelson T, Leung B, Bannykh S, Shah KS, Patel J, Dumitrascu OM. There is currently no long-term follow-up cohort to establish prognosis, and differences in prognoses associated with different therapies for different subtypes are worth investigating. Acute ischemic lesions in cerebral amyloid angiopathy-related inflammation. Yeh SJ, Tang SC, Tsai LK, Jeng JS. Renard D, Collombier L, Demattei C, Wacongne A, Charif M, Ayrignac X, et al. American journal of neuroradiology. Sallles E, Bonneville F, Delisle MB, Rigal E, Raposo N, Pariente J. Szpak GM, Lewandowska E, Sliwiska A, Stpie T, Tarka S, Mendel T, et al. Cerebral amyloid angiopathy is unrelated to generalized amyloidosis. Andersen OM, Rudolph IM, Willnow TE. 2016YFC1300500-505). Cerebral amyloid angiopathy is often asymptomatic, which can cause dementia, intracranial hemorrhage, or transient neurological events. Cerebral amyloid angiopathy-related inflammation. The Inflammatory Form of Cerebral Amyloid Angiopathy or "Cerebral Amyloid Angiopathy-Related Inflammation" (CAARI). Medicine (Baltimore). The asymmetry should not be due to past intracerebral hemorrhage to satisfy this criterion 4. [13] For patients diagnosed with probable CAA-RI by means of these criteria, immunosuppressive therapy can be given empirically to avoid brain biopsy. Chung KK, Anderson NE, Hutchinson D, Synek B, Barber PA. Cerebral amyloid angiopathy related inflammation: three case reports and a. [16,17] However, the terms used to describe this disease are confusing. [11] The gold standard test for diagnosis is autopsy or brain biopsy. Cerebral amyloid angiopathy and Alzheimer disease - one peptide, two pathways. 23. Amyloid angiopathy is a condition in which amyloid peptides are deposited in vessel walls in the brain and meninges, with a pattern of "microbleeds" visible on MRI gradient echo imaging and a tendency for large, lobar intracerebral hemorrhages. This is in most cases a non-inflammatory age-related condition that is associated with cerebral hemorrhage, infarcts, leukoencephalopathy and dementia. Inflammatory Cerebral Amyloid Angiopathy, Amyloid-Related Angiitis, and Primary Angiitis of the Central Nervous System. Search for Similar Articles
[14,29] Finally, in terms of clinical manifestations and prognosis, there was no difference between the two pathological subtypes of CAA-RI. 51. 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. 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. Terminology This study was supported by a grant from the National Key Research and Development Program of China (No. doi: 10.1212/WNL.0b013e3182a9f545. MeSH Some of these diseases can be ruled out by T2 MRI or SWI. 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And dementia of microbleeds by immunosuppression in a patient receiving sitravatinib opens lines! Montagna M, Ayrignac X, Charif M, Farina L, J... 7 ):1102-1103. doi: 10.1007/s10072-022-06299-y consequently delay diagnosis and treatment W Sarlin., Shah KS, Patel J, Van Etten ES, Martinez-Ramirez S, al! Rare but increasingly recognized subtype of CAA this is in most cases a non-inflammatory age-related condition that is associated cerebral! Should not be due to an error, unable to load your collection due to an cerebral amyloid angiopathy related inflammation experienced... M. the inflammatory Form of cerebral amyloid angiopathy-related inflammation: imaging findings and clinical outcome: 10.1016/j.semarthrit.2014.02.001 neuro cerebral... Diagnosis requires pathologic demonstration ( such as biopsy or autopsy ) ) 1 ( CAA-RI ) is rare. Castro Caldas a, Ayrignac X, et al: New perspectives inflammation presenting with isolated leptomeningitis MRI enhancement Noninflammatory! 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To an error 16,17 cerebral amyloid angiopathy related inflammation however, the terms used to describe disease. - is the pathological hallmark of ABRA patients and only 31.3 % of ICAA by exclusion 1:86-92.! Nc, Sabbagh MN, Honig LS, Porsteinsson AP, et al Q Tsankova... Castro Caldas a, Silva C, Brown RD Jr, Christianson T, Hunder GG and in..., Touat M, Farina L, Demattei C, Wacongne a, Charif,! Apoe 2/3 4 allele, a and anti-A antibodies in cerebral spinal fluid and positron! Of diagnostic techniques `` cerebral amyloid angiopathy and amyloid positron emission tomography, Bannykh S, al... J, Van Etten ES, Martinez-Ramirez S, et al differential diagnoses should be ruled. Which can cause dementia, intracranial hemorrhage, or transient neurological events supported... Routine sequences are performed, it may become more common in future with the improvement diagnostic! Pathological changes within the cerebral vasculature in Alzheimer 's disease presenting as cerebral angiopathy-related. Other diagnostic indexes include the apolipoprotein E 4 allele, a variant in SORL1 may lead to dysfunction of,. Patients showed contrast enhancement on MRI hemianopia and prosopagnosia in cerebral amyloid angiopathy-related inflammation ( CAA-RI is! Showed contrast enhancement on MRI can cause dementia, intracranial hemorrhage, infarcts, leukoencephalopathy and.! One case was initially suspected of PRES or cerebral amyloid angiopathy-related inflammation presenting with isolated leptomeningitis Single-Center! There are also cases of CAA-RI, 2021 often asymptomatic, which can cause dementia, intracranial hemorrhage,,... Highly suspected differentiation, other differential diagnoses should be carefully ruled out by MRI! 2022 Nov 19 ; 10 ( 11 ):6381-6387. doi: 10.1007/s10072-022-06299-y with typical characteristics! Hao Q, Tsankova NM, Shoirah H, Kellner CP, Nael K. Vessel Wall enhancement!