Kotsenas AL, Morris JM, Wald JT, Parisi JE, Campeau NG. In addition, the treatment of infection and other comorbidities should be considered in such cases. Imaging Findings of Cerebral Amyloid Angiopathy, A-Related Angiitis (ABRA), and Cerebral Amyloid Angiopathy-Related Inflammation: A Single-Institution 25-Year Experience. Department of Neurology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China. Salvarani C, Hunder GG, Morris JM, Brown RD, Christianson T, Giannini C. A-related angiitis: comparison with CAA without inflammation and primary CNS vasculitis. 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. Gera A, Witek N, Bailey M. Pearls & Oy-sters: CAA-related inflammation presents as subacute cognitive decline in a patient with Parkinson disease. Your message has been successfully sent to your colleague. Rajczewska-Oleszkiewicz C, Cyganek A, Stadnik A, Dziewulska D. Cerebral amyloid angiopathy-related inflammation - a case report presenting diagnostic difficulties. Terminology 55. 12. Diagnosis, treatment, and follow-up of patients with cerebral amyloid angiopathy-related inflammation. 10: 984. In general, the same patient group affected by cerebral amyloid angiopathy is affected, and thus most patients are elderly, typically 60-80 years of age. 49. 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]. The resultant vascular fragility tends to manifest in normotensive elderly patients as lobar intracerebral hemorrhage. In patients who respond to treatment, imaging follow-up demonstrates regression of the aforementioned inflammatory findings. 9. The possible mechanism is that APOE 4 increases A deposition, and has a pro-inflammatory effect. [19] Spontaneous remission has been reported in some cases,[7,71] the fundamentals of which are not yet known. [18] No difference in outcome was found between patients receiving mono-therapy of corticosteroid and patients receiving a combination of immunosuppressant and corticosteroid therapy. Brain Pathol. 1. There have been few epidemiological studies on CAA-RI. modify the keyword list to augment your search. Rapid progressive dementia, headache, seizures, or focal neurological deficits, with patchy or confluent hyperintensity on T2 or fluid-attenuated inversion recovery sequences and evidence of strictly lobar microbleeds or cortical superficial siderosis on susceptibility-weighted imaging imply CAA-RI. An official website of the United States government. The diagnostic criteria for possible or probable inflammatory cerebral amyloid angiopathy require age 40 years 4. This pathological distinction is not reliably predicted on imaging 2. Pathogenetical subtypes of recurrent intracerebral hemorrhage: designations by SMASH-U classification system. (E) No significant changes with CMBs. [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. Neuroradiology. 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. [10,42,43] SWI is considered to be more reliable than T2 imaging, with greater reliability and sensitivity for detection of CMBs. Ann Neurol 2013; 73:449. 10. Pseudotumoral presentation of cerebral amyloid angiopathy-related inflammation. 2022 Nov 14;11(22):6731. doi: 10.3390/jcm11226731. The former represents the inflammatory form of CAA, while the latter is an independent disease or a subtype of PACNS associated with CAA. doi: 10.1016/j.jstrokecerebrovasdis.2015.04.042. Inflammatory Cerebral Amyloid Angiopathy, Amyloid-Related Angiitis, and Primary Angiitis of the Central Nervous System. [65] Therefore, these two diseases are sometimes difficult to distinguish, and it may be necessary to observe changes during follow-up to obtain the correct diagnosis. Epub 2019 May 25. 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). Yeh SJ, Tang SC, Tsai LK, Jeng JS. Martucci M, Sarria S, Toledo M et-al. Cerebral amyloid--related angiitis without cerebral microbleeds in a patient with subarachnoid hemorrhage. The biopsy result revealed intravascular large B-cell lymphoma. Medicine (Baltimore). Mendona MD, Caetano A, Pinto M, Cruz e Silva V, Viana-Baptista M. Stroke-like episodes heralding a reversible encephalopathy: microbleeds as the key to the diagnosis of cerebral amyloid angiopathy-related inflammation-a case report and literature. Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare but increasingly recognized subtype of CAA. National Library of Medicine Aghetti A, Sene D, Polivka M, Shor N, Lechtman S, Chabriat H, et al. Biomedicines. Xu YY, Chen S, Zhao JH, Chen XL, Zhang JW. [64] Another patient was first diagnosed with PRES, which was responsive to anti-edema intravenous steroid and antihypertensive therapy. Radiographics. 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. 20. (2020) AJNR. Bogner S, Bernreuther C, Matschke J, Barrera-Ocampo A, Sepulveda-Falla D, Leypoldt F, et al. Epub 2022 Aug 5. See this image and copyright information in PMC. [17] Steroid therapy is also effective during recurrence, but increased microbleeds may be detected with T2/SWI sequences in that case. [9,10] Two pathological subtypes are now generally accepted: non-destructive perivascular inflammation (inflammatory CAA [ICAA]) and transmural or intramural inflammation (A-related angiitis [ABRA]). Inflammatory Disorders of the Central Nervous System Vessels: Narrative Review. Kirshner HS, Bradshaw M. The inflammatory form of cerebral amyloid angiopathy or cerebral amyloid angiopathy-related inflammation (CAARI). [17] While another systematic review showed that the functional outcome of most patients was not ideal. Miller-Thomas MM, Sipe AL, Benzinger TL et-al. 1 Introduction of the imaging-based Boston criteria for diagnosis of CAA in the 1990s 2, 3 (E) No significant changes with CMBs. Still others refer to only cerebral amyloid angiopathy-related inflammation alone 1,4,5,10or amyloid -related angiitis alone 7without mention of the other. official website and that any information you provide is encrypted 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]. This highlights the significance of the T2/SWI sequences in differentiation. Thus, PACNS is on the list of differential diagnoses whenever multifocal hyperintensity is seen on FLAIR images, although it is a diagnosis of exclusion. The site is secure. Melzer N, Harder A, Gross CC, Wolfer J, Stummer W, Niederstadt T, et al. Course of cerebral amyloid angiopathy-related inflammation. Cerebral amyloid angiopathy-related inflammation (CAAri) is characterized by vasogenic edema and multiple cortical/subcortical microbleeds, sharing several aspects with the recently defined amyloidrelated imaging abnormalities (ARIA) reported in Alzheimer's disease (AD) passive immunization therapies. 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. Kang P, Bucelli RC, Ferguson CJ, Corbo JC, Kim AH, Day GS. Renard D, Tatu L, Collombier L, Wacongne A, Ayrignac X, Charif M, et al. 32. [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. J Stroke 2015; 17:1730. A study has shown that more patients with ABRA (33.0%) require a combination of steroids and immunosuppressants than do patients with ICAA (12.8%), to achieve similar outcomes. 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. By definition, CAA is characterized by vessel wall amyloid deposits. Therefore, other biomarkers are needed to enrich the criteria. Since the treatment does not obviously harm the tumor, the response of the lesion to the given treatment can be observed to figure out whether it deteriorates as time goes by. HHS Vulnerability Disclosure, Help It is worth noting that CAA-RI is a diagnosis by exclusion. Renard D, Wacongne A, Ayrignac X, Charif M, Fourcade G, Azakri S, et al. 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. 19. Ng DW, Magaki S, Terashima KH, Keener AM, Salamon N, Karnezis S, et al. In autopsy series, the estimated prevalence of CAA is high (20-40 % in nondemented subjects; 50-60 % in dementia) [1]. It also remains unclear what should be done for those diagnosed with possible CAA-RI, and whether they still need to undergo brain biopsy. In addition, when starting the treatment, infection needs to be ruled out first, to avoid pervasion due to corticosteroid therapy. Epub 2014 Feb 11. 62. First, ABRA has the same radiological characteristics as ICAA, which are not common in PACNS. 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. 48. Cerebrospinal fluid Alzheimer's disease biomarkers in cerebral amyloid angiopathy-related inflammation. An alternative transcript of the Alzheimer's disease risk gene SORL1 encodes a truncated receptor. 10. [14], Angio-destructive changes, such as fibrinoid necrosis can also be found in some of the vessel walls in patients affected by ABRA. Diagnosis, treatment, and follow-up of patients with cerebral amyloid angiopathy-related inflammation. [13] Nevertheless, these criteria are still imperfect, as samples included in the validation trial was small. A Report of 2 Cases. Savoiardo M, Erbetta A, Storchi G, Girotti F. Case 159: cerebral amyloid angiopathy-related inflammation. DiFrancesco JC, Brioschi M, Brighina L, Ruffmann C, Saracchi E, Costantino G, et al. CMBs: Cerebral microbleeds; WMH: White matter hyperintensity. A 62-year-old man presented with a moderately severe non-radiating frontal headache. 46. Hao Q, Tsankova NM, Shoirah H, Kellner CP, Nael K. Vessel Wall MRI Enhancement in Noninflammatory Cerebral Amyloid Angiopathy. That is, 50% of all cases showed overlap between ICAA and ABRA patterns. Hemorrhage and white matter injury seen at imaging reflect vascular damage caused by the accumulation of A in vessel walls. Cerebral Amyloid Angiopathy-Related Inflammation: A Single-Center Experience and a Literature Review. Liang JW, Zhang W, Sarlin J, Boniece I. [47,60] In the future, the significance of these indicators for the differential diagnosis of CAA-RI mimics should be studied. Impact of A40 and A42 Fibrils on the Transcriptome of Primary Astrocytes and Microglia. Federal government websites often end in .gov or .mil. 6. Cerebral amyloid angiopathy (CAA) is a vasculopathy caused by deposition of amyloid (A) in the arteries and veins of the leptomeninges and cortex. 13. Perivascular and vascular inflammatory patterns without granulomas accounted for 22.5% of cases. 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. MRI is the modality of choice in assessing these patients as it is able to visualize the characteristic peripheral microhemorrhages of cerebral amyloid angiopathy. CAA-RI consists of two subtypes: inflammatory cerebral amyloid angiopathy and amyloid (A)-related angiitis. CMBs: Cerebral microbleeds; WMH: White matter hyperintensity. Bookshelf Unauthorized use of these marks is strictly prohibited. Moreover, ABRA was considered to be different from ICAA because it has the same vascular destructive pathological changes as PACNS. 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). Acta Neuropathol 1974; 27:131137. 2019 Sep-Oct;42:36-40. doi: 10.1016/j.carpath.2019.05.004. sharing sensitive information, make sure youre on a federal 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. A is deposited segmentally, but can be found in all those inflammation sites. 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. Finally, a multi-center prospective cohort study, using unified standards for the collection of data, application of designed therapies, and follow-up strategy is necessary. J Alzheimers Dis. [14] The recurrence probability of CAA-RI has differed across studies. (2015) Stroke. [11] The most commonly used immunosuppressants are cyclophosphamide (33.9%), azathioprine (5.0%), mycophenolate mofetil (5.0%), methotrexate, immunoglobulin, and so on. In humans, cerebral amyloid angiopathy and related vascular dysfunction are suggested to affect small vessels in the cortical areas [30,31]. Angiography does not reveal evidence of vasculitis involving the large- or medium-sized vessels 6. doi: 10.1161/strokeaha.114.005598. Cerebral amyloid angiopathy (CAA) is characterized by amyloid beta-peptide deposits within small- to medium-sized blood vessels of the brain and leptomeninges. Cerebrospinal fluid, MRI, and florbetaben-PET in cerebral amyloid angiopathy-related inflammation. 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