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A case of unexpected death due to primary angiitis of the central nervous system

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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.

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Abbreviations

PACNS:

Primary angiitis of the central nervous system

CT:

Computed tomography

MRI:

Magnetic resonance imaging

DSA:

Digital subtraction angiography

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Authors and Affiliations

Authors

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.

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Correspondence to Jianbo Li or Renkuan Tang.

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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

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