Transcranial Doppler Ultrasound in Children with Sturge-Weber Syndrome

Abstract

Transcranial Doppler ultrasound is a noninvasive vascular assessment technique proved useful in the management of pediatric disorders predisposed to stroke and may have similar utility for Sturge-Weber syndrome. Eight children with Sturge-Weber syndrome had velocities measured in the major cerebral arteries via the Stroke Prevention Trial in Sickle Cell Anemia methodology. Velocities and pulsatility indexes were compared between the unaffected and affected sides. All subjects had reduced velocity on the affected side; the mean middle cerebral artery percentage difference was 20% (95% CI, 15%-25%). Pulsatility index was increased on the affected side; mean middle cerebral artery pulsatility index percentage difference, 34% (95% CI, 15%-53%). Six subjects also had reduced posterior cerebral artery velocity on the affected side. Side-to-side differences in middle cerebral artery and posterior cerebral artery velocities correlated with severity of MRI asymmetry (Spearman rho = 0.88, P = .02). Decreased arterial flow velocity and increased pulsatility index in the middle cerebral artery and posterior cerebral artery suggests a high resistance pattern that may reflect venous stasis and may contribute to chronic hypoperfusion of brain tissue. Further study of Transcranial Doppler in children with Sturge-Weber syndrome is indicated.

Updates and future horizons on the understanding, diagnosis, and treatment of Sturge–Weber syndrome brain involvement

“…91 On the side affected by venous angioma, they had decreased arterial flow velocity and increased pulsatility index in the middle and posterior cerebral arteries; these measurements suggested a high resistance pattern of blood flow. 91 Since the pulsatility index measures the resistance to blood flow within a vessel, the higher the pulsatility index, the higher the downstream resistance to flow in the blood vessels. 92 One possibility is that this TCD pattern may reflect venous stasis within the venous angioma, which may contribute to chronic hypoperfusion of brain tissue.…”

“…42 Comparing TCD data from children with SWS to measurements made in healthy children suggests that children with SWS have a significantly more asymmetric blood flow. 91,94,95 …”

Use of quantitative EEG in infants with port-wine birthmark to assess for Sturge–Weber brain involvement

“…Experience in our center indicates that CT findings typically occur after MRI involvement is seen. Transcranial doppler studies have been looked at in Sturge-Weber syndrome (Jordan et al, 2008) but have not yet been investigated as a diagnostic modality.…”

Leveraging a Sturge-Weber Gene Discovery: An Agenda for Future Research

“…The BVMC trial has been collecting patient and family history as well as basic clinical data, both longitudinal and cross-sectional, since its inception in 2010. Biomarker development has also been a focus of this work with pilot biomarker studies completed and published, which have included the development of quantitative EEG 8;26;27 , transcranial Doppler 28 , medical rehabilitation 29 outcome measures, and urine biomarkers 30 seeking predictors of neurologic outcome and outcome measures for clinical trials. However, to date only some of these studies have been longitudinal and prospective (urine biomarkers ongoing and, to some extent, quantitative EEG), and more long-term outcome data is needed.…”