Brain Advance Access published online on November 27, 2008
Brain, doi:10.1093/brain/awn313
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The course and outcome of unilateral intracranial arteriopathy in 79 children with ischaemic stroke
1Department of Child Neurology, Rudolf Magnus Institute of Neuroscience, University Medical Center Utrecht, The Netherlands, 2Service de Pédiatrie, Hôpital Nord, CHU de Saint Etienne, France, 3UCL Institute of Child Health and Southampton University Hospitals NHS Trust, UK, 4Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, The Netherlands, 5Service de Neurologie Pédiatrique, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, France and 6Service de Neuropédiatrie, Centre Hospitalier Universitaire de Sherbrooke, Canada
Correspondence to:
K. P. J. Braun, MD, PhD, Department of Child Neurology, Rudolf Magnus Institute of Neuroscience, Wilhelmina Children's Hospital, University Medical Center Utrecht, PO Box 85500, 3508 GA Utrecht, The Netherlands E-mail: k.braun{at}umcutrecht.nl
Arteriopathies are the commonest cause of arterial ischaemic stroke (AIS) in children. Repeated vascular imaging in children with AIS demonstrated the existence of a ‘transient cerebral arteriopathy’ (TCA), characterized by lenticulostriate infarction due to non-progressive unilateral arterial disease affecting the supraclinoid internal carotid artery and its proximal branches. To further characterize the course of childhood arteriopathies, and to differentiate TCA from progressive arterial disease, we studied the long-term evolution of unilateral anterior circulation arteriopathy, and explored predictors of stroke outcome and recurrence. From three consecutive cohorts in London, Paris and Utrecht, we reviewed radiological studies and clinical charts of 79 previously healthy children with anterior circulation AIS and unilateral intracranial arteriopathy of the internal carotid bifurcation, who underwent repeated vascular imaging. The long-term evolution of arteriopathy was classified as progressive or TCA. Clinical and imaging characteristics were compared between both groups. Logistic regression modelling was used to determine possible predictors of the course of arteriopathy, functional outcome and recurrence. After a median follow-up of 1.4 years, 5 of 79 children (6%) had progressive arteriopathy, with increasing unilateral disease or bilateral involvement. In the others (94%), the course of arteriopathy was classified as TCA. In 23% of TCA patients, follow-up vascular imaging showed complete normalization, the remaining 77% had residual arterial abnormalities, with improvement in 45% and stabilization in 32%. Stroke was preceded by chickenpox in 44% of TCA patients, and in none of the patients with progressive arteriopathies. Most infarcts were localized in the basal ganglia. In 14 (19%) of TCA patients, transient worsening of the arterial lesion was demonstrated before the arteriopathy stabilized or improved. Thirteen TCA patients (18%) had a recurrent stroke or TIA. Thirty TCA patients (41%) had a good neurological outcome, compared with none of the five patients with progressive arteriopathy. Arterial occlusion, moyamoya vessels and ACA involvement were more frequent in progressive arteriopathies. Cortical infarct localization was significantly associated with poor neurological outcome (OR 6.14, 95% CI 1.29–29.22, P = 0.02), while there was a trend for occlusive arterial disease to predict poor outcome (OR 3.00, 95% CI 0.98–9.23, P = 0.06). Progressive arteriopathy was associated with recurrence (OR 18.77, 95%CI 1.94–181.97, P = 0.01). The majority of childhood unilateral intracranial anterior circulation arteriopathies (94%) have a course that is consistent with TCA, in which transient worsening is common. Although the arterial inflammation probably causing TCA is ‘transient’, most children are left with permanent arterial abnormalities and residual neurological deficits.
Key Words: arterial ischaemic stroke; children; transient cerebral arteriopathy; recurrence; outcome
Abbreviations: AIS, arterial ischaemic stroke; (d)ICA, (distal) internal carotid artery; DSA, digital subtraction contrast angiography; (p)ACA, (proximal) anterior cerebral artery; (p)MCA, (proximal) middle cerebral artery; PACNS, primary angiitis of the central nervous system; PVA, post-varicella angiopathy; TCA, transient cerebral arteriopathy; VZV, varicella zoster virus
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Received May 2, 2008. Revised October 2, 2008. Accepted October 31, 2008.
*These authors contributed equally to this work.