TY - JOUR
T1 - Reliability and Clinical Correlation of Transcranial Doppler Ultrasound in Sturge-Weber Syndrome
AU - National Institute of Health Sponsor
AU - Rare Disease Clinical Research Consortium (RDCRN)
AU - Brain and Vascular Malformation Consortium (BVMC)
AU - National Sturge-Weber Syndrome Workgroup
AU - Rare Disease Clinical Research Consortium (RDCRN)
AU - Brain and Vascular Malformation Consortium (BVMC)
AU - National Sturge-Weber Syndrome Workgroup
AU - Brain and Vascular Malformation Consortium (BVMC)
AU - National Sturge-Weber Syndrome Workgroup
AU - National Sturge-Weber Syndrome Workgroup
AU - Offermann, Elizabeth A.
AU - Sreenivasan, Aditya
AU - DeJong, M. Robert
AU - Lin, Doris D.M.
AU - McCulloch, Charles E.
AU - Chung, Melissa G.
AU - Comi, Anne M.
AU - Ball, Karen L.
AU - Fisher, Brian J.
AU - Hammill, Adrienne
AU - Juhász, Csaba
AU - Koenig, Jim
AU - Lawton, Michael
AU - Lo, Warren
AU - Marchuk, Douglas
AU - Miles, Daniel
AU - Moses, Marsha
AU - Wilfong, Angus
N1 - Publisher Copyright:
© 2017
PY - 2017/9
Y1 - 2017/9
N2 - Background The reproducibility of transcranial Doppler (TCD) ultrasound measurements in Sturge-Weber syndrome (SWS) and TCD's ability to predict neurological progression is unknown. Methods In 14 individuals with SWS, TCD measured mean flow velocity, pulsatility index, peak systolic velocity, and end-diastolic velocity in the middle, posterior, and anterior cerebral arteries of the affected and unaffected hemisphere. TCD was performed either once (n = 5) or twice in one day (n = 9). We assessed the reproducibility of the measurements performed twice on the same day on subjects and compared the TCD measurements to previously published age-matched controls. Clinically obtained neuroimaging was scored for extent and severity of SWS brain involvement. Patients were prospectively assigned SWS neuroscores. Results Middle cerebral artery velocity (r = 0.79, P = 0.04, n = 7), posterior cerebral artery velocity (r = 0.90, P = 0.04, n = 5), and anterior cerebral artery pulsatility index (r = 0.82, P = 0.02, n = 7) were reproducible TCD measurements comparing same-day percent side-to-side differences. In subjects with SWS, affected and unaffected mean peak systolic velocity and end-diastolic velocity in the middle, posterior, and anterior cerebral arteries were globally lower compared with age-matched control subjects. Subjects with the lowest affected middle cerebral artery velocity had the greatest worsening in the total neurological score between time 1 and 2 (r = −0.73, P = 0.04, n = 8) and the most severe magnetic resonance imaging involvement of the affected frontal lobe (r = −0.82, P = 0.007, n = 9). Conclusions TCD may be a reliable measure with potential clinical value, indicating that blood flow may be globally decreased in SWS patients with unilateral brain involvement.
AB - Background The reproducibility of transcranial Doppler (TCD) ultrasound measurements in Sturge-Weber syndrome (SWS) and TCD's ability to predict neurological progression is unknown. Methods In 14 individuals with SWS, TCD measured mean flow velocity, pulsatility index, peak systolic velocity, and end-diastolic velocity in the middle, posterior, and anterior cerebral arteries of the affected and unaffected hemisphere. TCD was performed either once (n = 5) or twice in one day (n = 9). We assessed the reproducibility of the measurements performed twice on the same day on subjects and compared the TCD measurements to previously published age-matched controls. Clinically obtained neuroimaging was scored for extent and severity of SWS brain involvement. Patients were prospectively assigned SWS neuroscores. Results Middle cerebral artery velocity (r = 0.79, P = 0.04, n = 7), posterior cerebral artery velocity (r = 0.90, P = 0.04, n = 5), and anterior cerebral artery pulsatility index (r = 0.82, P = 0.02, n = 7) were reproducible TCD measurements comparing same-day percent side-to-side differences. In subjects with SWS, affected and unaffected mean peak systolic velocity and end-diastolic velocity in the middle, posterior, and anterior cerebral arteries were globally lower compared with age-matched control subjects. Subjects with the lowest affected middle cerebral artery velocity had the greatest worsening in the total neurological score between time 1 and 2 (r = −0.73, P = 0.04, n = 8) and the most severe magnetic resonance imaging involvement of the affected frontal lobe (r = −0.82, P = 0.007, n = 9). Conclusions TCD may be a reliable measure with potential clinical value, indicating that blood flow may be globally decreased in SWS patients with unilateral brain involvement.
KW - Sturge-Weber syndrome
KW - end-diastolic velocity
KW - peak systolic velocity
KW - transcranial Doppler ultrasound
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U2 - 10.1016/j.pediatrneurol.2017.04.026
DO - 10.1016/j.pediatrneurol.2017.04.026
M3 - Article
C2 - 28757309
AN - SCOPUS:85026228211
SN - 0887-8994
VL - 74
SP - 15-23.e5
JO - Pediatric Neurology
JF - Pediatric Neurology
ER -