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Volume 13
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10.3390/brainsci13091336
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Review

Inflammatory Mechanisms in a Neurovascular Disease: Cerebral Cavernous Malformation

by
Ying Li
1,2,†,
Abhinav Srinath
2,†,
Roberto J. Alcazar-Felix
2,
Stephanie Hage
2,
Akash Bindal
2,
Rhonda Lightle
2,
Robert Shenkar
2,
Changbin Shi
1,‡,
Romuald Girard
2,‡ and
Issam A. Awad
2,3,*,‡
1
Department of Neurosurgery, First Affiliated Hospital of Harbin Medical University, Harbin 150001, China
2
Neurovascular Surgery Program, Department of Neurological Surgery, The University of Chicago, Chicago, IL 60637, USA
3
Department of Neurological Surgery, University of Chicago Medicine, 5841 S Maryland, MC3026/Neurosurgery J341, Chicago, IL 60637, USA
*
Author to whom correspondence should be addressed.
†
These authors contributed equally to this work.
‡
These authors contributed equally to this work.
Brain Sci. 2023, 13(9), 1336; https://doi.org/10.3390/brainsci13091336
Submission received: 21 July 2023 / Revised: 6 September 2023 / Accepted: 11 September 2023 / Published: 17 September 2023
(This article belongs to the Special Issue Cerebrovascular Neurosurgery)
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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.
Keywords: cerebral cavernous malformation; immune response; inflammation; transcriptome; immunothrombosis cerebral cavernous malformation; immune response; inflammation; transcriptome; immunothrombosis
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MDPI and ACS Style

Li, Y.; Srinath, A.; Alcazar-Felix, R.J.; Hage, S.; Bindal, A.; Lightle, R.; Shenkar, R.; Shi, C.; Girard, R.; Awad, I.A. Inflammatory Mechanisms in a Neurovascular Disease: Cerebral Cavernous Malformation. Brain Sci. 2023, 13, 1336. https://doi.org/10.3390/brainsci13091336

AMA Style

Li Y, Srinath A, Alcazar-Felix RJ, Hage S, Bindal A, Lightle R, Shenkar R, Shi C, Girard R, Awad IA. Inflammatory Mechanisms in a Neurovascular Disease: Cerebral Cavernous Malformation. Brain Sciences. 2023; 13(9):1336. https://doi.org/10.3390/brainsci13091336

Chicago/Turabian Style

Li, Ying, Abhinav Srinath, Roberto J. Alcazar-Felix, Stephanie Hage, Akash Bindal, Rhonda Lightle, Robert Shenkar, Changbin Shi, Romuald Girard, and Issam A. Awad. 2023. "Inflammatory Mechanisms in a Neurovascular Disease: Cerebral Cavernous Malformation" Brain Sciences 13, no. 9: 1336. https://doi.org/10.3390/brainsci13091336

APA Style

Li, Y., Srinath, A., Alcazar-Felix, R. J., Hage, S., Bindal, A., Lightle, R., Shenkar, R., Shi, C., Girard, R., & Awad, I. A. (2023). Inflammatory Mechanisms in a Neurovascular Disease: Cerebral Cavernous Malformation. Brain Sciences, 13(9), 1336. https://doi.org/10.3390/brainsci13091336

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