Multi-parametric magnetic resonance imaging evaluation of cerebral amyloid angiopathy related inflammation: Case series and review of literature

Nada, Ayman; Leiva‐Salinas, Carlos; Mahdi, Eman; Mahmoud, Eman; Ahsan, Humera; Cousins, Joseph P.

doi:10.1016/j.clinimag.2021.03.003

Clinical Imaging

2021

DOI: 10.1016/j.clinimag.2021.03.003

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Multi-parametric magnetic resonance imaging evaluation of cerebral amyloid angiopathy related inflammation: Case series and review of literature

Ayman Nada

Carlos Leiva‐Salinas

Eman Mahdi

et al.

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Cited by 3 publications

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“…4 Common neuroimaging characteristics in patients with CAA-ri include cerebral microbleeds (CMBs), unifocal or multifocal, cortical or subcortical, asymmetric T2/fluid-attenuated inversion recovery (FLAIR) hyperintense white matter lesions, lesions with gadolinium-enhancement, cortical superficial siderosis (cSS), spontaneous lobar intracerebral hemorrhage, and cortical infarcts. 5–7…”

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Clinical, Neuroimaging, and Genetic Markers in Cerebral Amyloid Angiopathy-Related Inflammation: A Systematic Review and Meta-Analysis

Theodorou

Palaiodimou

Malhotra

et al. 2023

Stroke

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BACKGROUND: There are limited data regarding the prevalence of distinct clinical, neuroimaging and genetic markers among patients diagnosed with cerebral amyloid angiopathy–related inflammation (CAA-ri). We sought to determine the prevalence of clinical, radiological, genetic and cerebrospinal fluid biomarker findings in patients with CAA-ri. METHODS: A systematic review and meta-analysis of published studies including patients with CAA-ri was conducted to determine the prevalence of clinical, neuroimaging, genetic and cerebrospinal fluid biomarker findings. Subgroup analyses were performed based on (1) prospective or retrospective study design and (2) CAA-ri diagnosis with or without available biopsy. We pooled the prevalence rates using random-effects models and assessed the heterogeneity using Cochran-Q and I 2 -statistics. RESULTS: We identified 4 prospective and 17 retrospective cohort studies comprising 378 patients with CAA-ri (mean age, 71.5 years; women, 52%). The pooled prevalence rates were as follows: cognitive decline at presentation 70% ([95% CI, 54%–84%]; I 2 =82%), focal neurological deficits 55% ([95% CI, 40%–70%]; I 2 =82%), encephalopathy 54% ([95% CI, 39%–68%]; I 2 =43%), seizures 37% ([95% CI, 27%–49%]; I 2 =65%), headache 31% ([95% CI, 22%–42%]; I 2 =58%), T2/fluid-attenuated inversion recovery-hyperintense white matter lesions 98% ([95% CI, 93%–100%]; I 2 =44%), lobar cerebral microbleeds 96% ([95% CI, 92%–99%]; I 2 =25%), gadolinium enhancing lesions 54% ([95% CI, 42%–66%]; I 2 =62%), cortical superficial siderosis 51% ([95% CI, 34%–68%]; I 2 =77%) and lobar macrohemorrhage 40% ([95% CI, 11%–73%]; I 2 =88%). The prevalence rate of the ApoE (Apolipoprotein E) ε4/ε4 genotype was 34% ([95% CI, 17%–53%]; I 2 =76%). Subgroup analyses demonstrated no differences in these prevalence rates based on study design and diagnostic strategy. CONCLUSIONS: Cognitive decline was the most common clinical feature. Hyperintense T2/fluid-attenuated inversion recovery white matter lesions and lobar cerebral microbleeds were by far the most prevalent neuroimaging findings. Thirty-four percent of patients with CAA-ri have homozygous ApoE ε4/ε4 genotype and scarce data exist regarding the cerebrospinal fluid biomarkers and its significance in these patients.

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Clinical, Neuroimaging, and Genetic Markers in Cerebral Amyloid Angiopathy-Related Inflammation: A Systematic Review and Meta-Analysis

Theodorou

Palaiodimou

Malhotra

et al. 2023

Stroke

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Neuroimaging to Facilitate Clinical Trials in Huntington’s Disease: Current Opinion from the EHDN Imaging Working Group

Hobbs,

Papoutsi,

Delva

et al. 2024

JHD

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Neuroimaging is increasingly being included in clinical trials of Huntington’s disease (HD) for a wide range of purposes from participant selection and safety monitoring, through to demonstration of disease modification. Selection of the appropriate modality and associated analysis tools requires careful consideration. On behalf of the EHDN Imaging Working Group, we present current opinion on the utility and future prospects for inclusion of neuroimaging in HD trials. Covering the key imaging modalities of structural-, functional- and diffusion- MRI, perfusion imaging, positron emission tomography, magnetic resonance spectroscopy, and magnetoencephalography, we address how neuroimaging can be used in HD trials to: 1) Aid patient selection, enrichment, stratification, and safety monitoring; 2) Demonstrate biodistribution, target engagement, and pharmacodynamics; 3) Provide evidence for disease modification; and 4) Understand brain re-organization following therapy. We also present the challenges of translating research methodology into clinical trial settings, including equipment requirements and cost, standardization of acquisition and analysis, patient burden and invasiveness, and interpretation of results. We conclude, that with appropriate consideration of modality, study design and analysis, imaging has huge potential to facilitate effective clinical trials in HD.

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Cerebral Amyloid Angiopathy—Related Inflammation: A Single-Center Experience and a Literature Review

Theodorou

Palaiodimou

Safouris

et al. 2022

JCM

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Background: Limited data exist regarding the prevalence of clinical, neuroimaging, and genetic markers among patients diagnosed with Cerebral Amyloid Angiopathy–related inflammation (CAA-ri). We sought to determine these characteristics in patients diagnosed in our center and to summarize available literature published either as single-case reports or small case series (<5 patients). Methods: We reported our single-center experience of patients diagnosed with CAA-ri according to international criteria during a seven-year period (2015–2022), and we abstracted data from 90 previously published cases. Results: Seven patients (43% women, mean age 70 ± 13 years) were diagnosed with CAA-ri in our center. The most common symptom at presentation was focal neurological dysfunction (71%), and the most prevalent radiological finding was the presence of T2/FLAIR white matter hyperintensities (100%). All patients were treated with corticosteroids and had a favorable functional outcome. Among 90 previously published CAA-ri cases (51% women, mean age 70 ± 9 years), focal neurological dysfunction was the most common symptom (76%), followed by a cognitive decline (46%) and headache (34%). The most prevalent neuroimaging findings were cerebral microbleeds (85%), asymmetric T2/FLAIR white matter hyperintensities (81%), and gadolinium-enhancing T1-lesions (37%). Genetic testing for the Apolipoprotein-E gene was available in 27 cases; 59% carried the APOE ε4/ε4 genotype. The majority of the published CAA-ri cases (78%) received corticosteroid monotherapy, while 17 patients (19%) were treated with additional immunosuppressive treatment. Favorable functional outcome following treatment was documented in 70% of patients. Conclusion: Improving the vigilance of clinicians regarding the early recognition and accurate diagnosis of CAA-ri is crucial for swift therapy initiation, which may result in improved functional outcomes.

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Multi-parametric magnetic resonance imaging evaluation of cerebral amyloid angiopathy related inflammation: Case series and review of literature

Cited by 3 publications

References 20 publications

Clinical, Neuroimaging, and Genetic Markers in Cerebral Amyloid Angiopathy-Related Inflammation: A Systematic Review and Meta-Analysis

Clinical, Neuroimaging, and Genetic Markers in Cerebral Amyloid Angiopathy-Related Inflammation: A Systematic Review and Meta-Analysis

Neuroimaging to Facilitate Clinical Trials in Huntington’s Disease: Current Opinion from the EHDN Imaging Working Group

Cerebral Amyloid Angiopathy—Related Inflammation: A Single-Center Experience and a Literature Review

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