Abstract
Background
Childhood primary angiitis of the central nervous system is a rare inflammatory disorder affecting the central nervous system in children. High-resolution magnetic resonance vessel wall imaging is less frequently used in the childhood primary angiitis of the central nervous system. This study aimed to investigate the value of high-resolution magnetic resonance vessel wall imaging in childhood primary angiitis of the central nervous system.
Methods
We retrospectively reviewed patients <18 years old who met the diagnostic criteria for primary angiitis of the central nervous system at Xiangya Hospital from January 2020 to April 2024. High-resolution magnetic resonance vessel wall imaging was performed to assess vessel wall enhancement, which was subsequently quantified for analysis.
Result
A total of 6 patients were included in the study, 3 of whom were male, with a mean age of onset of 10.3 years. The most common presenting symptoms were headache/dizziness and hemiplegia. Ischemic brain lesions were caused by stenosis of vessels in the anterior circulation in 4 patients and in the posterior circulation in 2 patients. Intravenous methylprednisolone was the primary immunotherapy used and was administered to all patients. None experienced relapse. A total of 174 arteries were evaluated, of which 38 exhibited grade 1 enhancement and 31 exhibited grade 2 enhancement. Bilateral internal carotid artery wall enhancement was noted in all patients. Corresponding offending arteries were identified in each case, all of which showed predominant enhancement and varying degrees of stenosis. The vessel wall enhancement score at the initial assessment was 16.7 ± 2.8, which significantly decreased to 12.7 ± 2.4 at the final assessment (P = .012).
Conclusion
High-resolution magnetic resonance vessel wall imaging appears to be a potentially effective tool for both diagnosing childhood primary angiitis of the central nervous system and monitoring response to treatment.
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