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Chapter 1.3 - Chapter

from 1 - Inflammatory Conditions

Published online by Cambridge University Press:  06 October 2022

Anita Arsovska
Affiliation:
University of Ss Cyril and Methodius
Derya Uluduz
Affiliation:
Istanbul Üniversitesi
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Summary

Cerebral Amyloid Angiopathy-related inflammation (CAA-ri) is a rare and sometimes aggressive autoimmune encephalopathy caused by immune reaction directed to the beta-amyloid protein in cerebral small vessels. It has been described in the main cerebral amyloid-related diseases, cerebral amyloid angiopathy (CAA) and Alzheimer’s Disease (AD) as a spontaneous occurrence and a phenomenon triggered by immunological therapy in recent trials for AD. Indeed there are several similarities between the clinic-radiological syndrome described in CAA (CAA-ri) and the neuroradiological phenomenon described in AD patients as Amyloid-Related Imaging Abnormalities (ARIA). CAA-ri is probably underdiagnosed because many CAA patients are elderly and cognitively impaired before CAA-ri occurrence. In some cases a missed diagnosis means a missed possibility of treatment because in its most striking form it is known as steroid-responsive dementia. The clinical expression of CAA-ri is characterized by the acute/subacute onset of neurological symptoms, accompanied by the neuroimaging evidence of cerebral vasogenic edema on FLAIR-MR sequences, and cortical-subcortical microbleeds (MBs) and/or cortical superficial siderosis (CSS) on T2*-weighted gradient echo (T2*-GRE) or susceptibility weighted imaging (SWI). CSS is a marker of CAA included in modified Boston criteria for CAA diagnosis and it represents a neuroradiological sign highly specific of CAA and associated to an even higher ICH risk than MBs. Atypical forms of CAA-RI have also been reported including radiologically isolated CAA-ri, minimally symptomatic CAA-RI, CAA-RI with isolated leptomeningitis, and CAA-RI without MBs. There is a well-defined set of clinic-radiological criteria, validated vs hysto-pathological examination with a sensitivity of 82% and a specificity of 97% for the category of probable CAA-ri. The main differential diagnosis is represented by neoplastic, infectious, or other even more rare causes

Type
Chapter
Information
Rare Causes of Stroke
A Handbook
, pp. 99 - 106
Publisher: Cambridge University Press
Print publication year: 2022

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  • Chapter
  • Edited by Derya Uluduz, Istanbul Üniversitesi
  • Anita Arsovska
  • Book: Rare Causes of Stroke
  • Online publication: 06 October 2022
  • Chapter DOI: https://doi.org/10.1017/9781108902793.018
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  • Chapter
  • Edited by Derya Uluduz, Istanbul Üniversitesi
  • Anita Arsovska
  • Book: Rare Causes of Stroke
  • Online publication: 06 October 2022
  • Chapter DOI: https://doi.org/10.1017/9781108902793.018
Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

  • Chapter
  • Edited by Derya Uluduz, Istanbul Üniversitesi
  • Anita Arsovska
  • Book: Rare Causes of Stroke
  • Online publication: 06 October 2022
  • Chapter DOI: https://doi.org/10.1017/9781108902793.018
Available formats
×