Spectrum of cerebral arteriopathies in children with arterial ischaemic stroke

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.