Introduction: Cerebral arteriopathies are frequently identified in children with arterial ischemic stroke (AIS), and are distinguished by high stroke recurrence. However, the clinical and neuroimaging profiles of AIS due to cerebral arteriopathy versus non- arteriopathy have not been compared.
Hypothesis: We hypothesized that children with AIS due to arteriopathy would differ in their demographic, clinical, and radiographic presentation from those without arteriopathy.
Methods: We report a large, prospective, multicentre cohort of children, 1 month - 17 years, with AIS, enrolled in the International Pediatric Stroke Study, 2003-2014. Those with arteriopathy including focal cerebral arteriopathy, dissection, moyamoya, vasculitis and non-specific arteriopathies, were compared to those with non-arteriopathic stroke etiologies.
Results: Of 2127 children with AIS, 725(34%) had arteriopathy (58% male, mean age 8.1years). The remaining 1402(66%) had non-arteriopathic AIS. Arteriopathy was associated with older age, but not a specific gender or ethnicity. Geographic differences were observed. Children with arteriopathy were likely to present with hemiparesis, dysarthria, ataxia, headache, preceding/concurrent thromboembolic events and without seizures. Risk factors associated with arteriopathy included sickle anemia, head/neck trauma and lack of acute systemic disease. Radiological associations with arteriopathy included right sided unilateral or bilateral stroke, multiple infarcts and relative reduction in occurrence of intracranial hemorrhage. On multivariate analysis, headache (p=0.006), additional thromboembolic events (p=0.007), multiple infarcts (p=0.002) and lack of seizures (p=0.001) were independently associated with arteriopathy.
Conclusions: Specific clinical profiles are associated with arteriopathy in children with AIS and may guide the clinician in early diagnostic evaluations and management.