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Purpose: High-resolution vessel wall-magnetic resonance imaging (VW-MRI) has been used to detect regional vessel wall pathology, significantly contributing to the diagnosis of primary angiitis of the central nervous system (PACNS) from other arteriopathies. In this study, we aimed to describe three cases of PACNS initially presenting as acute ischemic stroke, diagnosed and followed up with VW-MRI. Methods: The medical records of three patients diagnosed with PACNS were retrospectively reviewed. We also reviewed the clinical application of VW-MRI in published articles for the pediatric cases since 2016. Results: The initial presenting symptoms were headache, diplopia, confused mentality, hemiplegia, dysarthria, and dizziness. All patients had acute infarction on brain MRI, with suspicion of vasculopathy on magnetic resonance angiography. VW-MRI revealed narrowing of vessels with concentric wall thickening and diffuse enhancement in the anterior cerebral artery (n=1), middle cerebral artery (n=1), posterior cerebral artery (n=2), lenticulostriate artery (n=1), anterior inferior cerebellar artery (n=1), and vertebral artery (n=1), suggestive of vascular wall pathology. After excluding the possible etiologies of vasculitis, the clinical diagnosis of PACNS was established. Three patients received high-dose steroid and cyclophosphamide therapy. Two patients were treated with long-term azathioprine based upon the findings of persistent vessel enhancement on VW-MRI. All patients were regularly followed up with VW-MRI for more than 1 year. We summarized the clinical and radiological features of the published pediatric cases using VW-MRI to date. Conclusion: High-resolution VW-MRI plays an important role in diagnosing childhood PACNS, as results correlate with disease activity.

annals of child neurology

The Value of High-Resolution Vessel Wall Magnetic Resonance Imaging in the Diagnosis and Management of Primary Angiitis of the Central Nervous System in Children