Nonprogressive unilateral intracranial arteriopathy in children with arterial ischemic stroke

The nonprogressive unilateral intracranial arteriopathy known as transient (focal) cerebral arteriopathy is not a well-recognized arteriopathy among practitioners of Korea and Japan, although it cannot be easily differentiated from early moyamoya disease. This review summarizes the nomenclature, pathophysiology, diagnostic evaluation, clinico-radiological features, and management of nonprogressive (reversible or stable) unilateral arteriopathy based on the relevant literature and our own experiences. Nonprogressive unilateral arteriopathy should be strongly suspected in children presenting with basal ganglia infarction and arterial beading. The early identification of patients likely to have nonprogressive or progressive arteriopathy would ensure proper management and guide further research for secondary stroke prevention.