A Clinico-Radiological Study of Cerebral Amyloid Angiopathy-Related Inflammation

Coulette, Sarah; Renard, Dimitri; Lehmann, Sylvain; Raposo, Nicolas; Arquizan, Caroline; Charif, Mahmoud; Thouvenot, Éric; Wacongne, Anne; Viguier, Alain; Bonneville, Fabrice; Allou, Thibaut; Boukriche, Yassine; Chiper, Laura; Fourcade, G.; Gabelle, Audrey; Ducros, Anne; Duflos, Claire; Labauge, Pierre; Champfleur, Nicolas Menjot de; Ayrignac, Xavier

doi:10.1159/000502832

Cited by 21 publications

(25 citation statements)

References 26 publications

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“…Headache and seizure are often shown 12 . Visual symptoms were also seen 12 . Our nationwide survey based on 16 cases of CAA‐ri showed that dementia and headache were the most frequently observed as initial manifestations 6 .…”

Section: Clinical Manifestations Of Caamentioning

confidence: 85%

“…In patients with CAA‐ri, the most frequent manifestations are acute or subacute‐onset cognitive impairment and behavioral changes 10,11 . Headache and seizure are often shown 12 . Visual symptoms were also seen 12 .…”

Section: Clinical Manifestations Of Caamentioning

confidence: 99%

“…2b). 11,12 Unifocal or multifocal white matter hyperintense lesions, which are asymmetric and extend to the immediately subcortical white matter, are required to make probable CAA‐ri diagnosis 29 . The presence of contrast enhancement in the cerebral cortices and leptomeninges is often shown (Fig.…”

Section: Laboratory and Imaging Findings Of Caa And Caa‐rimentioning

confidence: 99%

“…Corticosteroid and immunosuppressant treatments have been used to improve CAA‐ri 6,7,10,11,12,34 . However, there have been no randomized‐controlled studies to confirm treatment recommendations.…”

Section: Treatments and Prognosis Of Caa And Caa‐rimentioning

confidence: 99%

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Cerebral amyloid angiopathy‐related inflammation and dementia

Sakai

Yamada

2021

Clinical & Exp Neuroim

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Cerebral amyloid angiopathy (CAA) is a disorder characterized by deposition of amyloid on the leptomeningeal and cortical blood vessels. Sporadic amyloid β (Aβ)‐type CAA is the most common type of CAA. Although CAA is well known as a cause of recurrent cerebral lobar hemorrhage, inflammation and vasculitis (CAA‐related inflammation [CAA‐ri]) induced by deposited Aβ on the vessel walls is emerging as a treatable condition. Furthermore, dementia could develop regardless of the presence of Alzheimer's disease pathology. The estimated total number and prevalence of CAA‐ri in Japan was reported as follows: 170 and 0.13 per 100 000 population, respectively. The estimated total number of CAA‐related intracerebral hemorrhage was 5900. The crude prevalence rate of CAA‐related intracerebral hemorrhage was 4.64 per 100 000 population. The patients with CAA‐ri show acute or subacute‐onset cognitive impairment, behavioral changes and headache. Brain magnetic resonance imaging is a useful tool for the diagnosis of CAA‐ri, showing asymmetrical white matter abnormalities and occasional meningeal enhancement. Furthermore, elevation of anti‐Aβ antibodies and inflammatory markers in the cerebrospinal fluid would be useful for clinical diagnosis. Clinical diagnostic criteria were proposed; however, neuropathological examination obtained by brain biopsy is still warranted to confirm severe Aβ deposition and vasculopathic changes with lymphocytic infiltrations and/or granulomatous vasculitis. No validated protocol of treatment has been established. Nearly 80% of the patients with CAA‐ri improved after immunosuppressant therapy, including corticosteroid and cyclophosphamide. Early treatment is necessary to prevent the development of irreversible sequela in the brain.

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Section: Clinical Manifestations Of Caamentioning

confidence: 85%

Section: Clinical Manifestations Of Caamentioning

confidence: 99%

Section: Laboratory and Imaging Findings Of Caa And Caa‐rimentioning

confidence: 99%

Section: Treatments and Prognosis Of Caa And Caa‐rimentioning

confidence: 99%

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Cerebral amyloid angiopathy‐related inflammation and dementia

Sakai

Yamada

2021

Clinical & Exp Neuroim

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“…Despite an apparently favorable initial evolution after a prompt empirical immunosuppressive therapy [24] , CAA-ri is characterized by a poor prognosis attributed to different factors such as CAA-relapses, intracerebral hemorrhages (ICH) and infectious or vascular complications [25] , [26] .…”

Section: Introductionmentioning

confidence: 99%

Cerebral amyloid angiopathy related inflammation: A little known but not to be underestimated disease

Grasso

Castorani

Borreggine

et al. 2021

Radiology Case Reports

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Cerebral amyloid angiopathy related inflammation (CAA-ri) is a rare encephalopathy resulting from perivascular inflammation after β-βamyloid (A) deposition in cerebral vessels leading to progressive dementia, focal neurological signs, seizures and intracerebral hemorrhages. This condition is characterized on magnetic resonance imaging (MRI) by patchy or confluent T2/fluid attenuation inversion recovery (FLAIR) hyperintensities in the cortex and subcortical white matter located mainly in the same areas of pre-existing multiple microhemorrhages. In this report of 2 cases of “probable” CAA-ri women aged 71 and 68, we propose a review on the pathophysiological, clinical, radiological, therapeutic and prognostic aspects of this little-known and poor outcome condition. Even though an apparently favorable initial evolution after steroid and/or immunosuppressive treatment, CAA-ri course is unpredictable and often associated with low survival rates. We suggest the importance of timely and proper clinico-radiological evaluation in suspected CAA-ri cases, in order to start an appropriate treatment even without the brain biopsy.

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Association Between Immunosuppressive Treatment and Outcomes of Cerebral Amyloid Angiopathy–Related Inflammation

et al. 2020

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IMPORTANCE Cerebral amyloid angiopathy-related inflammation (CAA-ri), a distinct subtype of cerebral amyloid angiopathy, is characterized by an autoimmune reaction to cerebrovascular β-amyloid deposits. Outcomes and response to immunosuppressive therapy for CAA-ri are poorly understood. OBJECTIVE To identify clinical, neuroimaging, laboratory, pathologic, or treatment-related associations with outcomes after an episode of CAA-ri. DESIGN, SETTING, AND PARTICIPANTSA retrospective cohort study of prospectively identified individuals who presented from July 3, 1998, to November 27, 2017, with a median follow-up of 2.7 years (interquartile range, 1.0-5.5 years). The study included 48 consecutive patients with CAA-ri meeting diagnostic criteria who had at least 1 disease episode and subsequent outcome data. No patients refused or were excluded.EXPOSURES Prespecified candidate variables were immunosuppressive therapies, cerebrospinal fluid pleocytosis, magnetic resonance imaging findings of recent infarcts or contrast enhancement, and histopathologic evidence of vessel wall inflammation.MAIN OUTCOMES AND MEASURES Clinical improvement and worsening were defined by persistent changes in signs or symptoms, radiographic improvement by decreased subcortical foci of T2 hyperintensity or T1 enhancement, and radiographic worsening by increased subcortical T2 hyperintensity, T1 enhancement, or infarcts. Disease recurrence was defined as new-onset clinical symptoms associated with new imaging findings. RESULTSThe 48 individuals in the study included 29 women and had a mean (SD) age of 68.9 (9.9) years. Results of presenting magnetic resonance imaging revealed that 10 of 29 patients with CAA-ri (34%) had T1 contrast enhancement, 30 of 32 (94%) had subcortical T2 hyperintensity (22 of 30 [73%] asymmetric), 7 of 32 (22%) had acute or subacute punctate infarcts, and 27 of 31 (87%) had microbleeds. Immunosuppressive treatments after first episodes included corticosteroids (33 [69%]), cyclophosphamide (6 [13%]), and mycophenolate (2 [4%]); 14 patients (29%) received no treatment. Clinical improvement and radiographic improvement were each more likely in individuals treated with an immunosuppressive agent than with no treatment (clinical improvement: 32 of 34 [94%] vs 7 of 14 [50%]; odds ratio, 16.0; 95% CI, 2.72-94.1; radiographic improvement: 24 of 28 [86%] vs 4 of 14 [29%]; odds ratio, 15.0; 95% CI, 3.12-72.1). Recurrence was less likely if CAA-ri was treated with any immunosuppressant agent than not (9 of 34 [26%] vs 10 of 14 [71%]; hazard ratio, 0.19; 95% CI, 0.07-0.48). When controlling for treatment, no variables were associated with outcomes aside from an association between APOE ε4 and radiographic improvement (odds ratio, 4.49; 95% CI, 1.11-18.2). CONCLUSIONS AND RELEVANCEThese results from a relatively large series of patients with CAA-ri support the effectiveness of immunosuppressive treatment and suggest that early treatment may both improve the initial disease course and reduce the likelihood of recurrence. These results r...

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A Clinico-Radiological Study of Cerebral Amyloid Angiopathy-Related Inflammation

Cited by 21 publications

References 26 publications

Cerebral amyloid angiopathy‐related inflammation and dementia

Cerebral amyloid angiopathy‐related inflammation and dementia

Cerebral amyloid angiopathy related inflammation: A little known but not to be underestimated disease

Association Between Immunosuppressive Treatment and Outcomes of Cerebral Amyloid Angiopathy–Related Inflammation

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