Spectrum of cerebral arteriopathies in children with arterial ischemic stroke
Mubeen F Rafay1, Kevin A Shapiro2, Ann-Marie Surmava2
1From the Section of Pediatric Neurology (M.F.R.), Department of Pediatrics and Child Health, University of Manitoba, Children's Hospital Research Institute of Manitoba, Winnipeg, Canada; Department of Neurology and Pediatrics (K.A.S., H.J.F.), University of California, San Francisco; University of Toronto (A.-M.S.); Division of Neurology (G.A.d.V., N.D., A.L.), The Hospital for Sick Children, University of Toronto, Ontario; Department of Pediatrics and Clinical Neurosciences (A.K.), Cumming School of Medicine, University of Calgary, Alberta, Canada; Department of Neurology (C.A.-L.), University of Washington, Seattle; Department of Neuropediatrics (B.W.), Charité University Medicine Berlin, Germany; Department of Neurology (J.L.C.), George Washington University, Washington, DC; Department of Neurology (M.T.M.), Royal Children's Hospital Melbourne, Murdoch Children's Research Institute and University of Melbourne, Australia; Boston Children's Hospital (M.R.), Harvard Medical School, Boston, MA; and Division of Child Neurology (T.J.B.), Department of Pediatrics and the Hemophilia and Thrombosis Center, University of Colorado, Denver. mubeen.rafay@utoronto.ca.
Insights
Children with arterial ischemic stroke (AIS) and arteriopathy have distinct clinical features. Identifying specific arteriopathy subtypes aids in early diagnosis and targeted treatments for pediatric stroke.
Area of Science:
- Pediatric Neurology
- Vascular Neurology
- Pediatric Stroke Research
Background:
- Arterial ischemic stroke (AIS) in children is a significant cause of long-term disability.
- Identifying the underlying causes of pediatric AIS is crucial for effective management.
- Arteriopathy, a disease of the arteries, is a known cause of AIS in children.
Purpose of the Study:
- To differentiate children with AIS due to arteriopathy from those without.
- To identify unique clinical features associated with each arteriopathy subtype in pediatric AIS.
- To establish distinct profiles for guiding diagnosis and treatment of pediatric arterial ischemic stroke.
Main Methods:
- Analysis of a large, observational, multicenter cohort of children (1 month to 18 years) with AIS.
- Comparison of clinical and demographic data using Fisher exact test and permutation adjustments.
- Exploratory analyses to evaluate differences between AIS with and without arteriopathy and between subtypes.
Main Results:
- Of 2,127 children with AIS, 725 (34%) had arteriopathy.
- Arteriopathy subtypes included dissection, moyamoya, focal cerebral arteriopathy-inflammatory (FCA-i), diffuse cerebral vasculitis, and nonspecific arteriopathy.
- Children with arteriopathic AIS were more likely to present with headache, multiple infarctions, and have a history of trauma or sickle cell anemia.
Conclusions:
- Distinct clinical profiles are associated with specific cerebral arteriopathies in children with AIS.
- These findings can aid in the early diagnosis of pediatric arterial ischemic stroke.
- Subgroup identification may facilitate targeted therapeutic trials for pediatric stroke.
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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