BACKGROUND AND PURPOSE: The nonprogressive, often reversible, unilateral arteriopathy known as transient (focal) cerebral arteriopathy has become a leading cause of childhood arterial ischemic stroke. However, it is not a well-recognized arteriopathy in East Asian countries where moyamoya disease is prevalent. METHODS: We retrospectively reviewed 74 children and adolescents (<18 years) with arterial ischemic stroke and intracranial arteriopathy to identify 29 patients with unilateral large-artery arteriopathy mainly in the anterior circulation. Among them, 25 patients who fulfilled the following inclusion criteria were analyzed to determine the angiographic course and outcome: (1) repeated vascular imaging at least twice and (2) absence of thrombotic disorders or cardiac diseases. RESULTS: The course of unilateral arteriopathy was classified as reversible in 17 patients (68%), progressive in 5 (20%), and stable in 3 (12%). Nine of the 17 patients with reversible arteriopathy exhibited initial worsening of the arteriopathy mostly within 1 month, but the worsened arteriopathy began to improve within 3 months and continued to improve even after a few years. Two of these 9 patients experienced stroke progression at 6 days. Of the variables analyzed, infarction involving the basal ganglia (15 of 17) and arterial beading on angiography performed within 2 weeks (10 of 12) were associated with reversible arteriopathy. Involvement of the ipsilateral posterior cerebral artery was rare (1 of 17). CONCLUSIONS: The possibility of reversible arteriopathy should be suspected in children and adolescents presenting with arterial ischemic stroke and unilateral arteriopathy.
BACKGROUND AND PURPOSE: The nonprogressive, often reversible, unilateral arteriopathy known as transient (focal) cerebral arteriopathy has become a leading cause of childhood arterial ischemic stroke. However, it is not a well-recognized arteriopathy in East Asian countries where moyamoya disease is prevalent. METHODS: We retrospectively reviewed 74 children and adolescents (<18 years) with arterial ischemic stroke and intracranial arteriopathy to identify 29 patients with unilateral large-artery arteriopathy mainly in the anterior circulation. Among them, 25 patients who fulfilled the following inclusion criteria were analyzed to determine the angiographic course and outcome: (1) repeated vascular imaging at least twice and (2) absence of thrombotic disorders or cardiac diseases. RESULTS: The course of unilateral arteriopathy was classified as reversible in 17 patients (68%), progressive in 5 (20%), and stable in 3 (12%). Nine of the 17 patients with reversible arteriopathy exhibited initial worsening of the arteriopathy mostly within 1 month, but the worsened arteriopathy began to improve within 3 months and continued to improve even after a few years. Two of these 9 patients experienced stroke progression at 6 days. Of the variables analyzed, infarction involving the basal ganglia (15 of 17) and arterial beading on angiography performed within 2 weeks (10 of 12) were associated with reversible arteriopathy. Involvement of the ipsilateral posterior cerebral artery was rare (1 of 17). CONCLUSIONS: The possibility of reversible arteriopathy should be suspected in children and adolescents presenting with arterial ischemic stroke and unilateral arteriopathy.