Reliability and Clinical Correlation of Transcranial Doppler Ultrasound in Sturge-Weber Syndrome

National Institute of Health Sponsor

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)15-23.e5
JournalPediatric Neurology
Volume74
DOIs
StatePublished - Sep 1 2017
Externally publishedYes

Fingerprint

Sturge-Weber Syndrome
Doppler Ultrasonography
Middle Cerebral Artery
Posterior Cerebral Artery
Anterior Cerebral Artery
Brain
Frontal Lobe
Neuroimaging
Magnetic Resonance Imaging

Keywords

  • end-diastolic velocity
  • peak systolic velocity
  • Sturge-Weber syndrome
  • transcranial Doppler ultrasound

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Developmental Neuroscience
  • Clinical Neurology

Cite this

Reliability and Clinical Correlation of Transcranial Doppler Ultrasound in Sturge-Weber Syndrome. / National Institute of Health Sponsor.

In: Pediatric Neurology, Vol. 74, 01.09.2017, p. 15-23.e5.

Research output: Contribution to journalArticle

National Institute of Health Sponsor. / Reliability and Clinical Correlation of Transcranial Doppler Ultrasound in Sturge-Weber Syndrome. In: Pediatric Neurology. 2017 ; Vol. 74. pp. 15-23.e5.
@article{9f1c131e305d452283dc760cf8a8b9d7,
title = "Reliability and Clinical Correlation of Transcranial Doppler Ultrasound in Sturge-Weber Syndrome",
abstract = "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.",
keywords = "end-diastolic velocity, peak systolic velocity, Sturge-Weber syndrome, transcranial Doppler ultrasound",
author = "{National Institute of Health Sponsor} and Offermann, {Elizabeth A.} and Aditya Sreenivasan and DeJong, {M. Robert} and Doris Lin and McCulloch, {Charles E.} and Chung, {Melissa G.} and Comi, {Anne Marie Spalding} and Ball, {Karen L.} and Fisher, {Brian J.} and Adrienne Hammill and Csaba Juh{\'a}sz and Jim Koenig and Michael Lawton and Warren Lo and Douglas Marchuk and Daniel Miles and Marsha Moses and Angus Wilfong",
year = "2017",
month = "9",
day = "1",
doi = "10.1016/j.pediatrneurol.2017.04.026",
language = "English (US)",
volume = "74",
pages = "15--23.e5",
journal = "Pediatric Neurology",
issn = "0887-8994",
publisher = "Elsevier Inc.",

}

TY - JOUR

T1 - Reliability and Clinical Correlation of Transcranial Doppler Ultrasound in Sturge-Weber Syndrome

AU - National Institute of Health Sponsor

AU - Offermann, Elizabeth A.

AU - Sreenivasan, Aditya

AU - DeJong, M. Robert

AU - Lin, Doris

AU - McCulloch, Charles E.

AU - Chung, Melissa G.

AU - Comi, Anne Marie Spalding

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

PY - 2017/9/1

Y1 - 2017/9/1

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 - end-diastolic velocity

KW - peak systolic velocity

KW - Sturge-Weber syndrome

KW - transcranial Doppler ultrasound

UR - http://www.scopus.com/inward/record.url?scp=85026228211&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85026228211&partnerID=8YFLogxK

U2 - 10.1016/j.pediatrneurol.2017.04.026

DO - 10.1016/j.pediatrneurol.2017.04.026

M3 - Article

C2 - 28757309

AN - SCOPUS:85026228211

VL - 74

SP - 15-23.e5

JO - Pediatric Neurology

JF - Pediatric Neurology

SN - 0887-8994

ER -