Abstract
Background
Focal cerebral arteriopathy (FCA) is a common cause of childhood arterial ischemic
stroke in previously healthy children. Although its mechanisms are poorly understood,
recent studies have suggested inflammatory processes. Magnetic resonance vessel wall
imaging (VWI) is a potential imaging biomarker of inflammation.
Case description: We describe the case of a 7-year-old Japanese girl with right hemiplegia
and dysarthria for 3 days. Brain MRI showed acute infarct in the left basal ganglia,
and MRA and conventional cerebral angiogram detected vascular stenosis in the left
distal internal carotid artery, left M1 and A1 segments. VWI revealed marked vessel
wall enhancement and thickening in the left carotid artery, M1, and A2 segments. Based
on imaging findings, she was diagnosed with acute ischemic stroke caused by FCA. Because
VWI findings were thought to suggest vessel wall inflammation, high-dose steroid therapy
was administered in addition to neuroprotective care and antithrombotic therapy. Although
her clinical symptoms improved immediately, cerebral arteriopathy worsened on MRA
a month after the onset. Subsequently, after 3 months of steroid therapy, vessel wall
enhancement on VWI decreased, while arterial stenosis partially improved. At the follow-up
9 months after the onset, she had no recurrent stroke, her arteriopathy had stabilized.
Discussion
Definitive evidence of inflammatory mechanisms in FCA is limited, and appropriate
management and treatment strategies for FCA are undefined. VWI attempts to demonstrate
pathologic processes within the vessel wall, and reversible wall enhancement observed
in our patient suggested the presence of inflammation. VWI would help in the evaluation
of disease activity in FCA.
Conclusion
VWI may contribute to the appropriate diagnosis and treatment for FCA to reflect active
inflammation. Further work is needed to assess the utility of VWI in pediatric FCA.
Keywords
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Article info
Publication history
Published online: February 17, 2023
Accepted:
February 5,
2023
Received in revised form:
January 22,
2023
Received:
May 30,
2022
Publication stage
In Press Corrected ProofIdentification
Copyright
© 2023 The Authors. Publishing services by Elsevier B.V. on behalf of KeAi Communications Co. Ltd. All rights reserved.
