Objective: To determine that children with arterial ischaemic stroke 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 into the International Pediatric Stroke Study from 2003 to 2014. Clinical and demographic differences were compared using the Fisher exact test, with linear step-up permutation min-p adjustment for multiple comparisons. Exploratory analyses were conducted to evaluate differences between AIS cases 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 non-specific arteriopathy (18.5%). Children with arteriopathic AIS were more likely to present between 6-9 years of age (p=0.029, OR 1.93), with headache (p=0.023, OR 1.55), multiple infarctions (p<0.001, OR 2.05), sickle cell anemia (p=0.007, OR 2.9), and head/neck trauma (p=0.018, OR 1.93). Antithrombotic usage and stroke recurrence were higher in children with arteriopathy. Among arteriopathy subtypes, patient with dissection were associated with male sex, older age, headache, and anticoagulant use; FCA-i with hemiparesis and single infarcts; moyamoya with seizures and recurrent strokes; and vasculitis 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 that may benefit most from future therapeutic trials.