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

Autor: Offermann, Elizabeth A, Sreenivasan, Aditya, DeJong, M Robert, Lin, Doris DM, McCulloch, Charles E, Chung, Melissa G, Comi, Anne M, National Institute of Health Sponsor, Rare Disease Clinical Research Consortium (RDCRN), Brain and Vascular Malformation Consortium (BVMC), National Sturge-Weber Syndrome Workgroup
Rok vydání: 2017
Předmět:
Popis: BackgroundThe reproducibility of transcranial Doppler (TCD)ultrasound measurements in Sturge-Weber syndrome (SWS)and TCD's ability to predict neurological progression is unknown.MethodsIn 14 individuals with SWS, TCD measured mean flow velocity, pulsatility index, peak systolic velocity, and end-diastolic velocityin the middle, posterior, and anterior cerebral arteriesof 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.ResultsMiddle 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).ConclusionsTCD may be a reliable measure with potential clinical value, indicating that blood flow may be globally decreased in SWS patients with unilateral brain involvement.
Databáze: OpenAIRE