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

Publication date: Available online 8 May 2017
Source:Pediatric Neurology
Author(s): Elizabeth A. Offermann, Aditya Sreenivasan, M. Robert DeJong, Doris D.M. Lin, Charles E. McCulloch, Melissa G. Chung, Anne M. Comi
BackgroundThe reproducibility of transcranial Doppler (TCD) ultrasound measurements in Sturge-Weber syndrome (SWS) and TCD's ability to predict neurologic progression is unknown.MethodsIn fourteen SWS patients, TCD measured mean flow velocity, pulsatility index, peak systolic velocity (PSV), and end diastolic velocity (EDV) in the middle (MCA), posterior (PCA) and anterior cerebral arteries (ACA) 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.ResultsMCA velocity (r=0.79, p=0.04, n=7), PCA velocity (r=0.90, p=0.04, n=5), and ACA 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 PSV and EDV velocities in the MCA, PCA, and ACA were globally lower compared to age-matched controls. Subjects with the lowest affected MCA velocity had the greatest worsening in total neurologic score between time 1 and 2 (r=-0.73, p=0.04, n=8) and the most severe MRI involvement of the affected frontal lobe (r=-0.82, p=0.007, n=9).ConclusionsTCD is suggested as a reliable measure with potential clinical value, indicating blood flow may be globally decreased in SWS patients with unilateral brain involvement.



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