This study was supported by a grant from the National Key Research and Development Program of China (No. This disorder typically responds to steroids but addition of other immune suppressants may be needed in some cases to control the disease. Other diagnostic indexes include the apolipoprotein E 4 allele, A and anti-A antibodies in cerebral spinal fluid and amyloid positron emission tomography. This highlights the significance of the T2/SWI sequences in differentiation. 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. [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]. [12,14,18] The erythrocyte sedimentation rate was increased in 37.5% of patients, while C-reactive protein (CRP) was elevated in 60%. 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. This site needs JavaScript to work properly. [24] There are three current hypotheses: (1) coexistence of vascular A and vascular inflammation implies that A is a bystander of angiitis; (2) inflammation promotes accumulation of A in the vessel wall; (3) A deposition triggers the inflammatory response. However, biopsy is invasive; consequently, most clinically diagnosed cases have been based on clinical and radiological data. Terminology A case of cerebral amyloid angiopathy-related inflammation with the rare apolipoprotein epsilon2/epsilon2 genotype. In fact, in a subgroup of patients, spontaneous remission is encountered 1. CAA-RI consists of two subtypes: inflammatory cerebral amyloid angiopathy and amyloid (A)-related angiitis. Tumoral presentation of homonymous hemianopia and prosopagnosia in cerebral amyloid angiopathy-related inflammation. Brashear, H.M. Arrighi, K.A. [22,31] In fact, both ICAA and ABRA can present with or without granulomatous inflammation. 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. 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). (2013) American Journal of Neuroradiology. Thus, in this review, we present the main pathological, clinical, neuroimaging, therapeutic, and prognostic features and the diagnostic criteria of CAA-RI to shed some light on its clinical practice, and then discuss issues that remain unresolved. 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. These cases emphasize that CAA-RI is a diagnosis by exclusion. CAA-RI is now widely recognized as a relatively rare and aggressive subtype of CAA with diverse clinical presentations and characteristic radiological findings. 2016;51(2):525-32. doi: 10.3233/JAD-151036. Growing numbers of patients have been reported with vascular inflammation associated with advanced cerebral amyloid angiopathy (59; 150). Acute or subacute onset of cognitive decline or behavioral changes is the most common symptom of CAA-RI. may email you for journal alerts and information, but is committed {"url":"/signup-modal-props.json?lang=us"}, Gaillard F, Sharma R, Deng F, et al. That is, 50% of all cases showed overlap between ICAA and ABRA patterns. This is in most cases a non-inflammatory age-related condition that is associated with cerebral hemorrhage, infarcts, leukoencephalopathy and dementia. The presence of symmetric white matter lesions that extend to the immediately subcortical white matter would only meet the criteria for "possible" inflammatory cerebral amyloid angiopathy 4. The .gov means its official. 69. [47,60] In the future, the significance of these indicators for the differential diagnosis of CAA-RI mimics should be studied. Immunosuppressive therapy is effective both during initial presentation and in relapses. modify the keyword list to augment your search. Kotsenas AL, Morris JM, Wald JT, Parisi JE, Campeau NG. 51. CAA-RI consists of two subtypes: inflammatory cerebral amyloid angiopathy and amyloid (A)-related angiitis. Medicine (Baltimore). [54] Therefore, the presence of the APOE 4/4 genotype may be meaningful for the diagnosis of CAA-RI. BMC Neurol. After treatment with corticoids, (D) WMH faded significantly. Epub 2022 May 18. Cerebral amyloid--related angiitis without cerebral microbleeds in a patient with subarachnoid hemorrhage. The resultant vascular fragility tends to manifest in normotensive elderly patients as lobar intracerebral haemorrhage. Imaging Findings of Cerebral Amyloid Angiopathy, A-Related Angiitis (ABRA), and Cerebral Amyloid Angiopathy-Related Inflammation: A Single-Institution 25-Year Experience. Salvarani C, Hunder GG, Morris JM, Brown RD, Christianson T, Giannini C. A-related angiitis: comparison with CAA without inflammation and primary CNS vasculitis. [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. In sporadic CAA, vascular amyloid is composed of the same 39- to 43-amino acid A peptide observed in the neuritic plaques of Alzheimer's disease (AD). 2018;64(4):1113-1121. doi: 10.3233/JAD-180269. 11. Typical images of cerebral amyloid angiopathy-related inflammation. 68. 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. 6. 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. 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. . [5] Unlike non-inflammatory CAA, acute or subacute onset of cognitive decline or behavioral changes are the most common symptom of CAA-RI. However, many authors interchange the terms "cerebral amyloid angiopathy-related inflammation" and "inflammatory cerebral amyloid angiopathy," either encompassing of amyloid -related angiitis 8 or in distinction to it 3. In addition, some researchers found that, compared with non-inflammatory CAA, PACNS, and healthy controls, patients with CAA-RI have relatively low levels of A42 and A40 in the CSF. Thus, other differential diagnoses should be carefully ruled out. 23. 71. 59. (E) No significant changes with CMBs. 17. Nationwide survey on cerebral amyloid angiopathy in Japan. 36. Eng JA, Frosch MP, Choi K, Rebeck GW, Greenberg SM. Unable to load your collection due to an error, Unable to load your delegates due to an error. and transmitted securely. Stroke 2014; 45:26362642. and transmitted securely. 61. Other differential diagnoses include viral or autoimmune encephalitis, cerebral venous thrombosis, acute disseminated encephalomyelitis (ADEM), Hashimoto encephalopathy, neurosarcoidosis, and acute toxic-metabolic leukoencephalopathy. Anti-A autoantibodies in the CSF of a patient with CAA-related inflammation: a case report. The site is secure. A 62-year-old man presented with a moderately severe non-radiating frontal headache. A Report of 2 Cases. Cerebral amyloid angiopathy is an increasingly important cause of hemorrhagic strokes in older adults, contributing to the growing vascular . [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. It may also present with cognitive impairments, incidental . [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]. Brain MRI, particularly FLAIR and T2/SWI sequences, is the most important imaging modality for the identification of patients suspected of CAA-RI. At present, the main recommendation is that high-dose glucocorticoids should be used. Cerebral amyloid angiopathy and Alzheimer disease - one peptide, two pathways. However, many patients present with atypical symptoms other than those mentioned above, which may easily lead to an incorrect diagnosis. Pathological changes within the cerebral vasculature in Alzheimer's disease: New perspectives. In patients who respond to treatment, imaging follow-up demonstrates regression of the aforementioned inflammatory findings. 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. See this image and copyright information in PMC. 31. [15] In fact, these two types sometimes do coexist. 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. Some error has occurred while processing your request. Piazza F, Greenberg SM, Savoiardo M, Gardinetti M, Chiapparini L, Raicher I, et al. Although CAA-RI is relatively rare at present, it may become more common in future with the improvement of diagnostic techniques. The Karolinska Imaging Dementia Study. 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. 2016YFC1300500-505). Reid and Maloney first described CAA with vascular inflammation in a patient with AD in 1974, and subsequent cases were reported. Andersen OM, Rudolph IM, Willnow TE. Reduction of microbleeds by immunosuppression in a patient with A-related vascular inflammation. Other diagnostic indexes include the apolipoprotein E 4 allele, A and anti-A antibodies in cerebral spinal fluid and amyloid positron emission tomography. doi: 10.1111/bpa.13061. [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. MRI is the modality of choice in assessing these patients as it is able to visualize the characteristic peripheral microhemorrhages of cerebral amyloid angiopathy. your express consent. Dear Sirs, Cerebral amyloid angiopathy (CAA) causes intracerebral haemorrhages and is associated with cognitive impairment and Alzheimer's disease. Please try after some time. 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]. 55. Chin Med J 2021;134:646654. Neurol Clin Pract. The https:// ensures that you are connecting to the Cerebrospinal fluid, MRI, and florbetaben-PET in cerebral amyloid angiopathy-related inflammation. In order to make a diagnosis before histopathology, Chung et al[12] proposed the Boston criteria using clinicoradiological data in 2011. 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. -, Yeh SJ, Tang SC, Tsai LK, Jeng JS. Medicine (Baltimore). 14. 43. A 77-year-old female experienced light-headedness during walking and mild ataxic gait without any other objective neuropsychological deficits. Gera A, Witek N, Bailey M. Pearls & Oy-sters: CAA-related inflammation presents as subacute cognitive decline in a patient with Parkinson disease. The accuracy of the standard was verified, and yielded a sensitivity and specificity of 82% and 97% diagnosing probable CAA-RI, respectively. 33. First, ABRA has the same radiological characteristics as ICAA, which are not common in PACNS. Objective. Nakaya M, Hashimoto H, Usui G, Sawada K, Shirouzu I, Oshima A, et al. [58,59] Thus, a variant in SORL1 may lead to dysfunction of SorLA, eventually adding to the risk of CAA-RI. Cerebral amyloid angiopathy and cerebral amyloid angiopathy-related inflammation: comparison of hemorrhagic and DWI MRI features. Cerebral amyloid angiopathy-related inflammation with posterior reversible encephalopathy syndrome-like presentation: a case report. 8. Prodromal Alzheimer's disease presenting as cerebral amyloid angiopathy-related inflammation with spontaneous amyloid-related imaging abnormalities and high cerebrospinal fluid anti-A autoantibodies. Mandal J, Chung SA. [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. [10] The carriage rate in non-inflammatory CAA patients was only 5.1%,[10] and it is rarely seen in healthy people or stroke patients. 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