Abstract
Primary angiitis of the central nervous system (PACNS) is a rare and fatal cerebral vasculitis mainly involving the arteriole of the pia mater, cerebral cortex, and spinal cord. It has an insidious onset atypical symptoms. In this paper, we reported an unexpected death due to cerebral hemorrhage caused by PACNS. According to the typical clinical manifestations (headache, dizziness, weakness of the limbs, temporary blurred vision, etc.) and pathological examination (wide degeneration and fibrinoid necrosis of blood vessel walls with inflammatory cell infiltration), as well as hematoxylin–eosin staining, immunohistochemistry for CD15 + and gram staining, we finally determined that the patient died due to cerebral vascular rupture and hemorrhage caused by PACNS. This case illustrates the value and key points of autopsy in evaluating sudden deaths.
Similar content being viewed by others
Abbreviations
- PACNS:
-
Primary angiitis of the central nervous system
- CT:
-
Computed tomography
- MRI:
-
Magnetic resonance imaging
- DSA:
-
Digital subtraction angiography
References
Hajj-Ali RA, Calabrese LH. Primary angiitis of the central nervous system. Autoimmun Rev. 2013;12(4):463–6.
Kraemer M, Berlit P. Primary central nervous system vasculitis - an update on diagnosis, differential diagnosis and treatment. J Neurol Sci. 2021;424:117422.
Wang Q, Wang Z, Wang K, et al. Characteristics of mass lesion presentation of primary angiitis of the central nervous system: a single center 11-year retrospective case-series study. Clin Neurol Neurosurg. 2020;199:106297.
Bernstein JE, Podkovik S, Kashyap S, et al. Primary angiitis of the central nervous system presenting as a cerebral mass lesion: a case report and literature review. Cureus. 2020;12(6):e8511.
Rao NM, Prasad PS, Flippen CC 2nd, et al. Primary angiitis of the central nervous system presenting as unilateral optic neuritis. J Neuroophthalmol. 2014;34(4):380–5.
Wang LJ, Kong DZ, Guo ZN, et al. Study on the clinical, imaging, and pathological characteristics of 18 cases with primary central nervous system vasculitis. J Stroke Cerebrovasc Dis. 2019;28(4):920–8.
Calabrese LH, Mallek JA. Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore). 1988;67(1):20–39.
Murthy JMK, Challa S, Parida S, et al. Primary angiitis of central nervous system-tumor-like mass lesions: report of six biopsy confirmed cases. Neurol India. 2021;69(2):451–6.
Stoecklein VM, Kellert L, Patzig M, et al. Extended stereotactic brain biopsy in suspected primary central nervous system angiitis: good diagnostic accuracy and high safety. J Neurol. 2021;268(1):367–76.
Cho TA, Jones A. CNS vasculopathies: challenging mimickers of primary angiitis of the central nervous system. Best Pract Res Clin Rheumatol. 2020;34(4):101569.
Beelen J, Benseler SM, Dropol A, et al. Strategies for treatment of childhood primary angiitis of the central nervous system. Neurol Neuroimmunol Neuroinflamm. 2019;6(4):e567.
Dabas A, Yadav S. Primary angiitis of the central nervous system: a rare and reversible cause of childhood stroke. J Pediatr Neurosci. 2016;11(4):338–40.
Ogoke CC, Ike II, Osuji AE, et al. Multiple subacute bilateral cerebral infarcts in a 9-year-old Nigerian male: a case of childhood primary angiitis of the central nervous system. Pan Afr Med J. 2018;31:99.
Hassan AS, Trobe JD, McKeever PE, et al. Linear magnetic resonance enhancement and optic neuropathy in primary angiitis of the central nervous system. J Neuroophthalmol. 2003;23(2):127–31.
Alrawi A, Trobe JD, Blaivas M, et al. Brain biopsy in primary angiitis of the central nervous system. Neurology. 1999;53(4):858–60.
Hajj-Ali RA, Singhal AB, Benseler S, et al. Primary angiitis of the CNS. Lancet Neurol. 2011;10(6):561–72.
Alhalabi M, Moore PM. Serial angiography in isolated angiitis of the central nervous system. Neurology. 1994;44(7):1221–6.
Nishikawa M, Sakamoto H, Katsuyama J, et al. Multiple appearing and vanishing aneurysms: primary angiitis of the central nervous system: Case report. J Neurosurg. 1998;88(1):133–7.
Ilhan A, Budak F. Primary angiitis of the central nervous system: unusual clinical presentation. Int J Angiol. 2000;9(1):23–6.
Marone G, Spadaro G, De Marino V, et al. Immunopharmacology of human mast cells and basophils. Int J Clin Lab Res. 1998;28(1):12–22.
Inder SJ, Bobryshev YV, Cherian SM, et al. Immunophenotypic analysis of the aortic wall in Takayasu’s arteritis: involvement of lymphocytes, dendritic cells and granulocytes in immuno-inflammatory reactions. Cardiovasc Surg. 2000;8(2):141–8.
Younger DS. Overview of the vasculitides. Neurol Clin. 2019;37(2):171–200.
Author information
Authors and Affiliations
Contributions
Li Yang and Zhe Zheng conceived and designed the case report, collected all data, and wrote the manuscript. Li Yang, Yujie Zeng, Shangyu Zhang, and Pengyue Jin contributed to the pathology diagnosis, immunohistochemistry, and its photographic material. Yongguo Li and Zhe Zheng contributed to the pathology diagnosis and its photographic material. Jianbo Li and Renkuan Tang supervised the conception, analysis, design of the work, and manuscript drafting. All authors critically revised the manuscript for important intellectual content, provided approval of the final version, and agreed to be accountable for all aspects of the work, ensuring that questions related to the accuracy or integrity of all parts of the work are appropriately investigated and resolved.
Corresponding authors
Ethics declarations
Ethics approval
Written informed consent was obtained from the patient’s family.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
Yang, L., Zheng, Z., Zeng, Y. et al. A case of unexpected death due to primary angiitis of the central nervous system. Forensic Sci Med Pathol (2023). https://doi.org/10.1007/s12024-023-00631-4
Accepted:
Published:
DOI: https://doi.org/10.1007/s12024-023-00631-4