Background and purpose: Unilateral intracranial focal nonprogressive arteriopathy is often found in children with arterial ischemic stroke. We aimed to investigate the course of unilateral intracranial arteriopathy in young adults.
Methods: We searched the Utrecht Stroke Database for patients between 16 and 50 years of age diagnosed with anterior circulation arterial ischemic stroke and a nonatherosclerotic, unilateral intracranial large-artery arteriopathy between 1991 and 2005. We assessed clinical features, potential causes, risk factors, extent of infarction and arteriopathy at presentation, long-term angiographic course, and clinical outcome.
Results: Of 356 patients with anterior circulation arterial ischemic stroke, 17 (5%) had a documented unilateral intracranial arteriopathy, of whom 14 could be included for follow-up investigations (median age, 34 years; range, 27-49 years). Median duration of follow-up was 8.8 years (range, 1.7-12.8 years). In 11 patients, onset of symptoms was not abrupt. The arteriopathy normalized completely in 5 and improved in 3 patients; in none of the patients did the arteriopathy worsen. Two of 14 patients had recurrent symptoms. Ten patients (71%) had a good outcome (modified Rankin Scale score≤2).
Conclusions: In young adults, arterial ischemic stroke is rarely caused by a unilateral intracranial arteriopathy. Similar to children, onset of symptoms in young adults is often not abrupt and the arteriopathy may improve over time. Late recurrences were rare. Possibly, a monophasic inflammatory process, as has been suggested for childhood intracranial focal nonprogressive arteriopathies, also occurs in young adults.