Primary angiitis of the central nervous system:report of one case and review of literature
LIANG Feng-xian
LIU Nan
YAO Zhi-cheng
Abstract：Objective: To explore the clinical, imaging, and pathological features of primary angiitis of the central nervous system (PACNS). Methods: A case of PACNS was reported, including its clinical manifestations, imaging, and pathological characteristics, and related literature was reviewed. Results: The patient was a 29-year-old female with clinical manifestations including fever, headache, nausea and vomiting, visual impairment, bilateral facial muscle twitching, and gait instability. MRI showed widespread involvement of the cerebral cortex, subcortical, deep white matter, and corpus callosum, with multiple intracerebral hemorrhagic lesions. Histopathology: There was proliferation of histiocytes between the cerebral cortex and white matter, with foamy granular cytoplasm. Lymphocytes and neutrophils were observed around blood vessels, along with glial cell proliferation. Inflammatory cell infiltration was seen in the walls of small veins and microvessels. Immunohistochemistry: CD68 (perivascular histiocytes +), GFAP (proliferating glial cells +), Ki-67 (approximately 10% +), CD3 (perivascular lymphocytes +), CD20 (perivascular lymphocytes +). Conclusion: The clinical symptoms of PACNS are mild, but the imaging findings are severe. The final diagnosis of the disease requires integration of clinical manifestations, imaging, and pathology.
Keywords：Primary Angiitis of the Central Nervous SystemClinical ManifestationsImagingPathology
Publication Date：2025-06-28
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：5( 736-740 )