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    Spectrum of cerebral arteriopathies in children with arterial ischemic stroke

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    Author
    Rafay, MF; Shapiro, KA; Surmava, A-M; DeVeber, GA; Kirton, A; Fullerton, HJ; Amlie-Lefond, C; Weschke, B; Dlamini, N; Carpenter, JL; ...
    Date
    2020-06-09
    Source Title
    Neurology
    Publisher
    LIPPINCOTT WILLIAMS & WILKINS
    University of Melbourne Author/s
    Mackay, Mark
    Affiliation
    Paediatrics (RCH)
    Metadata
    Show full item record
    Document Type
    Journal Article
    Citations
    Rafay, M. F., Shapiro, K. A., Surmava, A. -M., DeVeber, G. A., Kirton, A., Fullerton, H. J., Amlie-Lefond, C., Weschke, B., Dlamini, N., Carpenter, J. L., Mackay, M. T., Rivkin, M., Linds, A. & Bernard, T. J. (2020). Spectrum of cerebral arteriopathies in children with arterial ischemic stroke. NEUROLOGY, 94 (23), pp.E2479-E2490. https://doi.org/10.1212/WNL.0000000000009557.
    Access Status
    Access this item via the Open Access location
    URI
    http://hdl.handle.net/11343/254089
    DOI
    10.1212/WNL.0000000000009557
    Open Access URL
    https://escholarship.org/content/qt3rt7g864/qt3rt7g864.pdf?t=qbnm6a
    Abstract
    OBJECTIVE: To determine that children with arterial ischemic stroke (AIS) due to an identifiable arteriopathy are distinct from those without arteriopathy and that each arteriopathy subtype has unique and recognizable clinical features. METHODS: We report a large, observational, multicenter cohort of children with AIS, age 1 month to 18 years, enrolled in the International Pediatric Stroke Study from 2003 to 2014. Clinical and demographic differences were compared by use of the Fisher exact test, with linear step-up permutation min-p adjustment for multiple comparisons. Exploratory analyses were conducted to evaluate differences between cases of AIS with and without arteriopathy and between arteriopathy subtypes. RESULTS: Of 2,127 children with AIS, 725 (34%) had arteriopathy (median age 7.45 years). Arteriopathy subtypes included dissection (27%), moyamoya (24.5%), focal cerebral arteriopathy-inflammatory subtype (FCA-i; 15%), diffuse cerebral vasculitis (15%), and nonspecific arteriopathy (18.5%). Children with arteriopathic AIS were more likely to present between 6 and 9 years of age (odds ratio [OR] 1.93, p = 0.029) with headache (OR 1.55, p = 0.023), multiple infarctions (OR 2.05, p < 0.001), sickle cell anemia (OR 2.9, p = 0.007), and head/neck trauma (OR 1.93, p = 0.018). Antithrombotic use and stroke recurrence were higher in children with arteriopathy. Among arteriopathy subtypes, dissection was associated with male sex, older age, headache, and anticoagulant use; FCA-i was associated with hemiparesis and single infarcts; moyamoya was associated with seizures and recurrent strokes; and vasculitis was associated with bilateral infarctions. CONCLUSION: Specific clinical profiles are associated with cerebral arteriopathies in children with AIS. These observations may be helpful indicators in guiding early diagnosis and defining subgroups who may benefit most from future therapeutic trials.

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