Rajczewska-Oleszkiewicz C, Cyganek A, Stadnik A, Dziewulska D. Cerebral amyloid angiopathy-related inflammation - a case report presenting diagnostic difficulties. Yamada M. Cerebral amyloid angiopathy: emerging concepts. [3] CAA related lobar ICH has been identified as the second most common form of spontaneous ICH following hypertensive angiopathy. Liang JW, Zhang W, Sarlin J, Boniece I. Unauthorized use of these marks is strictly prohibited. 12. 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. [14] The dosage used is based on individual selection. A spectrum from CAA to PACNS: pathological differences between CAA, ICAA, ABRA, and PACNS. Typical images of cerebral amyloid angiopathy-related inflammation. 1 Introduction of the imaging-based Boston criteria for diagnosis of CAA in the 1990s 2, 3 [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. The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). (2016) JAMA neurology. 57. Pathogenetical subtypes of recurrent intracerebral hemorrhage: designations by SMASH-U classification system. CAA-RI shares pathologic characteristics of CAA, which is A deposition in the cortical or leptomeningeal vessels, with positive Congo red staining. Sengoku R, Matsushima S, Murakami Y, Fukuda T, Tokumaru AM, Hashimoto M, et al. -, Salvarani C, Hunder GG, Morris JM, Brown RD, Christianson T, Giannini C. A-related angiitis: comparison with CAA without inflammation and primary CNS vasculitis. In patients who respond to treatment, imaging follow-up demonstrates regression of the aforementioned inflammatory findings. However, anticoagulation was later suspended due to cerebral hemorrhage, and the patient was finally diagnosed with CAA-RI. There have been few epidemiological studies on CAA-RI. DiFrancesco JC, Brioschi M, Brighina L, Ruffmann C, Saracchi E, Costantino G, et al. [33] Findings from several systematic reviews have shown that there is no obvious gender difference, but a slight male predominance was observed. Cerebral amyloid angiopathy-related inflammation: a case report presenting with a rare variant in SORL1 gene. The mechanism underlying CAA-RI remains unclear. Cerebral amyloid--related angiitis without cerebral microbleeds in a patient with subarachnoid hemorrhage. Cerebrospinal fluid, MRI, and florbetaben-PET in cerebral amyloid angiopathy-related inflammation. Wolters Kluwer Health
In an elderly patient with multiple white matter lesions and the appropriate clinical presentation, MR images depicting microhemorrhages may be the key to diagnosing cerebral amyloid angiopathy-related inflammation; finding the apolipoprotein E 4-4 genotype may strongly support the diagnosis. Sperling R, Salloway S, Brooks DJ, Tampieri D, Barakos J, Fox NC, et al. 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. 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. [47,60] In the future, the significance of these indicators for the differential diagnosis of CAA-RI mimics should be studied. Clinical history of progressive cognitive decline over a few weeks and asymmetrically grouped cerebral microbleeds with focal corticosubcortical FLAIR hyperintensity, untypical for stroke and without restricted diffusion, we suspected cerebral amyloid angiopathy related inflammation (CAA-RI). Blechingberg J, Poulsen ASA, Kjlby M, Monti G, Allen M, Ivarsen AK, et al. Cerebral amyloid angiopathy-related inflammation with posterior reversible encephalopathy syndrome-like presentation: a case report. 51. 68. Subcortical white matter will demonstrate usually a solitary area of low density with localized mass effect 1,2. [12,13] Because immunosuppressive therapy is effective for the disease, timely diagnosis and early commencement of therapy are very important. [46] Two-thirds of ABRA patients and only 31.3% of ICAA patients showed contrast enhancement on MRI. Acute or subacute onset of cognitive decline or behavioral changes is the most common symptom of CAA-RI. Inflammatory cerebral amyloid angiopathy is a largely reversible inflammatory vasculopathy that develops in an acute or subacute fashion in reaction to amyloid protein deposition in the central nervous system blood vessels. Kimura A, Sakurai T, Yoshikura N, et al. [55,56] Thus, 2 carriers may also be predisposed to CAA-RI. Brain Pathol. Traschtz A, Tzaridis T, Penner AH, Kuchelmeister K, Urbach H, Hattingen E, et al. The diagnostic criteria for both probable and possible inflammatory cerebral amyloid angiopathy require at least one corticosubcortical hemorrhagic lesion 4, which is best demonstrated as signal loss on T2*-weighted sequences (susceptibility-weightedor gradient echo): cerebral macrobleed (intraparenchymal hematoma), cerebral microbleed (cerebral microhemorrhage). Table 4. WMH and vasogenic edema accompanied by a mass effect make brain tumors a highly suspected differentiation. 55. 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. CAA-RI consists of two subtypes: inflammatory cerebral amyloid angiopathy and amyloid (A)-related angiitis. Probable Cerebral Amyloid Angiopathy-Related Inflammation Associated With Sitravatinib: A Case Report. 56. Cerebral amyloid angiopathy-related inflammation in the immunosuppressed: a case report. 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. Reduction of microbleeds by immunosuppression in a patient with A-related vascular inflammation. Therefore, other biomarkers are needed to enrich the criteria. Giant cell arteritis and arteriolitis associated with amyloid angiopathy in an elderly mongol. 7. [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. 2016 May;95(20):e3613. [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. American journal of neuroradiology. 2014 Aug;44(1):86-92. doi: 10.1016/j.semarthrit.2014.02.001. One case was initially suspected of PRES or cerebral venous sinus thrombosis and was treated with anticoagulant and steroid. In another case, the patient had clinical and imaging characteristics of CAA-RI, but because of bicytopenia and an increase in CRP and lactate dehydrogenase, lymphoma was suspected. Auriel E, Charidimou A, Gurol ME, Ni J, Van Etten ES, Martinez-Ramirez S, Boulouis G, Piazza F, DiFrancesco JC, Frosch MP, Pontes-Neto OV, Shoamanesh A, Reijmer Y, Vashkevich A, Ayres AM, Schwab KM, Viswanathan A, Greenberg SM. If only routine sequences are performed, it is easy to mistake WMH as the only image manifestation and consequently delay diagnosis and treatment. 63. 8. 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. 2022 Apr;12(2):e4-e6. Reid AH, Maloney AF. Thirteen percent of patients were affected with some forms of visual impairment. 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. 20. 1-6 It differs from more common noninflammatory forms of CAA . Impact of A40 and A42 Fibrils on the Transcriptome of Primary Astrocytes and Microglia. Neurol Clin Pract. 2018;64(4):1113-1121. doi: 10.3233/JAD-180269. An official website of the United States government. CT and MRI demonstrate an area of vasogenic edema involving the subcortical white matter 1. 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. Piazza F, Greenberg SM, Savoiardo M, Gardinetti M, Chiapparini L, Raicher I, et al. 7. 1. Saliou V, Ben Salem D, Ognard J, Guellec D, Marcorelles P, Rouhart F, et al. 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. Brain MRI 9 months later showed multiple discrete regions . CD4(+) T cells predominate in cerebrospinal fluid and leptomeningeal and parenchymal infiltrates in cerebral amyloid beta-related angiitis. There are still many questions related to CAA-RI that require investigation. This article reviews the pathology and pathogenesis, clinical and imaging manifestations, diagnostic criteria, treatment, and prognosis of CAA-RI, and highlights unsolved problems in the existing research. 2022 Nov;43(11):6381-6387. doi: 10.1007/s10072-022-06299-y. [12,14,18] The erythrocyte sedimentation rate was increased in 37.5% of patients, while C-reactive protein (CRP) was elevated in 60%. Pathological changes within the cerebral vasculature in Alzheimer's disease: New perspectives. Zhu X, Schrader JM, Irizarry BA, Smith SO, Van Nostrand WE. Giant cell arteritis and arteriolitis associated with amyloid angiopathy in an elderly mongol. In humans, cerebral amyloid angiopathy and related vascular dysfunction are suggested to affect small vessels in the cortical areas [30,31]. 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. Please enable it to take advantage of the complete set of features! Moreover, ABRA was considered to be different from ICAA because it has the same vascular destructive pathological changes as PACNS. The use of glucocorticoids and immunosuppressants improves prognosis. 2022 Nov 14;11(22):6731. doi: 10.3390/jcm11226731. [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. Both variants produce a clinical picture that resembles primary angiitis of the CNS but is distinguished by a characteristic radiologic appearance. [67] For such patients, a clinicoradiological diagnosis only may result in missing a coexisting tumor, and thus the pros and cons of biopsy should be weighed carefully. may email you for journal alerts and information, but is committed
Salvarani C, Hunder GG, Morris JM, Brown RD, Christianson T, Giannini C. A-related angiitis: comparison with CAA without inflammation and primary CNS vasculitis. Blood tests may reveal signs of inflammation. Acute ischemic lesions in cerebral amyloid angiopathy-related inflammation. This pathological distinction is not reliably predicted on imaging 2. The case of an 85-year-old female with acute right hemiparesis with status epilepticus. 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. 6. Salvarani C, Brown RD Jr, Calamia KT, Christianson TJ, Huston J 3rd, Meschia JF, et al. 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. [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. Case of cerebral amyloid angiopathy-related inflammation - is the absence of cerebral microbleeds a good prognostic sign? The resultant vascular fragility tends to manifest in normotensive elderly patients as lobar intracerebral haemorrhage. 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. Moosavi B, Torres C, Jansen G. Case 232: Amyloid -related Angiitis. Pseudotumoral presentation of cerebral amyloid angiopathy-related inflammation. The distribution of CMBs does not follow the regional pattern of occipital dominance in non-inflammatory CAA. 42. 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