Symptomatic focal cerebral arteriopathy in a young man

Abstract

Arteriopathy is the leading cause of acute ischemic stroke in children and young adults, with intracranial arterial dissection (IAD) and transient cerebral arteriopathy (TCA) most commonly reported. The etiology and pathophysiology of childhood cerebral arteriopathies are not completely understood. The diagnosis is mainly based on clinical presentation and neuroimaging findings with considerable overlapping and discrepancy between diagnoses. We report the case of a young man who presented with an acute right basal ganglia infarct, and was found to have a focal cerebral arteriopathy of the intracranial right internal carotid artery that fluctuated clinically but resolved in four weeks with conservative management. The diagnoses of IAD and TCA were considered. We review and discuss the literature and the difficulties with the current terminology and classifications. In order to better understand the natural history and management there is a need to standardize the terminology and classifications of cerebral arteriopathies of the children and young adults.