Inflammatory Mechanisms in a Neurovascular Disease: Cerebral Cavernous Malformation

Overview

Journal Brain Sci

Publisher MDPI

Date 2023 Sep 28

PMID 37759937

Authors

Ying Li

Abhinav Srinath

Roberto J Alcazar-Felix

Stephanie Hage

Akash Bindal

Rhonda Lightle

Robert Shenkar

Changbin Shi

Romuald Girard

Issam A Awad

Affiliations

Soon will be listed here.

Abstract

Cerebral cavernous malformation (CCM) is a common cerebrovascular malformation causing intracranial hemorrhage, seizures, and focal neurologic deficits. A unique CCM lesional inflammatory microenvironment has been shown to influence the clinical course of the disease. This review addresses the inflammatory cell infiltrate in the CCM lesion and the role of a defined antigen-driven immune response in pathogenicity. We summarize immune mechanisms associated with the loss of the CCM gene and disease progression, including the potential role of immunothrombosis. We also review evidence of circulating inflammatory biomarkers associated with CCM disease and its clinical activity. We articulate future directions for this research, including the role of individual cell type contributions to the immune response in CCM, single cell transcriptomics of inflammatory cells, biomarker development, and therapeutic implications. The concepts are applicable for developing diagnostic and treatment strategies for CCM and for studying other neurovascular diseases.

Citing Articles

Cerebral cavernous malformation with prolonged postoperative paralysis due to perilesional inflammation: illustrative case.

Inai S, Sano N, Takeuchi Y, Makino Y, Hattori E, Takada S J Neurosurg Case Lessons. 2024; 8(23).

PMID: 39622022 PMC: 11616148. DOI: 10.3171/CASE24570.

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