Neurology: What is the progress and prognosis of inflammation associated with cerebral amyloid angiopathy?

Cerebral amyloid vascular lesions associated inflammation (Cerebral amyloid angiopathy-related inflammation, CAA-ri) is a rare self immune encephalopathy, cortical and meningeal deposited in arteries, arterioles and capillaries amyloid wall against CSF β (Aβ) protein autoantibody concentration increases
.

Vascular Immunity

The disease is characterized by acute/subacute neurological symptoms, ranging from very mild cognitive impairment and headaches to rapidly progressing cognitive decline, seizures , changes in mental status, and focal neurological dysfunction
.

epilepsy

These neurological symptoms are related to the acute white matter hyperintensities (WMHs) that indicate angioedema (VE) on magnetic resonance imaging (MRI), and the MRI features of CAA such as cerebral microhemorrhage (CMBs) and superficial cortical spider web disease (cSS).
) Related
.

A clear diagnosis of CAA-ri requires histopathological confirmation
.

However, recent clinical radiology standards and ultra-sensitive tests for the detection of anti-Aβ autoantibodies in cerebrospinal fluid (CSF) have greatly increased the current diagnostic capabilities and reduced the need for invasive brain biopsy

diagnosis

There is evidence that CAA-ri is a potentially reversible disease that responds to immunosuppressive therapy, but this is limited to rare case reports or small retrospective case series from a single center
.

CAA-ri is a potentially reversible disease that responds to immunosuppressive therapy, but this is limited to rare case reports or small retrospective case series from a single center
.

Although CAA-ri is considered to be limited to CAA patients, the disease is now increasingly recognized and diagnosed as different neurological and neuroradiological manifestations, including in clinical immunological trials for Alzheimer's disease (AD) The striking similarity of the observed amyloid-related imaging abnormalities (ARIA)
.

Treatment with anti-Aβ monoclonal antibodies is complicated by the following conditions:
1) ARIA-E (edema), including WMH localized areas suggesting parenchymal edema and/or exudation,
2) ARIA-H (bleeding), including CMBs And cSS
.

In view of the very small number of samples and data in clinical trials, the spontaneous ARIA (ARIA-like) in CAA-ri is proposed as an effective model for the study of treatment-induced ARIA in such trials
.

This led to the establishment of a collaborative research framework, the "Inflammatory Cerebral Amyloid Angiopathy and Alzheimer's disease βiomarkers (iCAβ) International Network" initiative, which aims to In:

manage

In this way, Laura Antolini and others of the University of Milan, Italy, explored the natural history of CAA-ri and provided new evidence for the results related to corticosteroid therapy.

The study was systematically recruited through the iCAβ International Network Initiative, and the first diagnosis was CAA-ri is well-defined and carried out by patients

Explored the natural history of CAA-ri and provided new evidence for the results related to corticosteroid therapy,

Between January 2013 and March 2017, they recruited inpatients who met the diagnostic criteria of CAA-ri through the iCAβ international network.

This is a multi-center, hospital-based longitudinal, prospective observational study

The history of ICH and the occurrence of new ICH at follow-up were more common in patients with superficial cortical arachnoid disease at baseline (52.
6% vs 14.
3%, respectively; p<0.
0001 and 19.
3% vs 3.
6%; p<0.
009)
.

After the first appearance of CAA-ri, 70.
3% (95% CI, 61.
6-78.
5) and 84.
1% (95% CI, 76.
2-90.
6) recovered clinically within three months and twelve months, respectively, followed by 45.
1% (95% CI, 76.
2-90.
6).
%CI, 36.
4-54.
8) and 77.
4% (95%CI, 67.
7-85.
9) radiological rehabilitation
.

After the first episode of clinical radiology recovered, 38.
3% (95% CI, 22.
9-59.
2) had at least one relapse within the following 24 months
.

If the pulse therapy with high-dose intravenous corticosteroids is suddenly stopped, there is a greater chance of recurrence compared with slow oral dose reduction (hazard ratio 4.

These results from the largest longitudinal cohort registration of CAA-ri patients support the prevention of the transient and potentially recurring inflammatory nature of the clinical-radiological acute manifestations of the disease

Original source:
Antolini L, DiFrancesco JC, Zedde M, et al.

Spontaneous ARIA-like Events in Cerebral Amyloid Angiopathy–Related Inflammation: A Multicenter Prospective Longitudinal Cohort Study.
 Neurology.
Published online September 16, 2021:10.
1212/WNL.

Spontaneous ARIA-like Events in Cerebral Amyloid Angiopathy--Related Inflammation: A Multicenter Prospective Longitudinal Cohort Study.
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