BACKGROUND
The reproducibility of transcranial Doppler (TCD) ultrasound measurements in Sturge-Weber syndrome (SWS) and TCD’s ability to predict neurologic progression is unknown.
METHODS
In 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.
RESULTS
MCA 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).
CONCLUSIONS
TCD is suggested as a reliable measure with potential clinical value, indicating blood flow may be globally decreased in SWS patients with unilateral brain involvement.
Excessive acquiring is a common symptom of hoarding disorder (HD). Little is known about subjective distress associated with acquiring in HD. The present study examined acquiring-related distress and reactions to cognitive restructuring (CR) in 92 individuals with HD and 66 community control (CC) participants. All participants identified an item of interest at a high-risk acquiring location and then decided whether or not to acquire the item. HD participants completed the acquiring task while receiving a CR-based intervention or a thought-listing (TL) control condition. Results showed that HD participants reported more severe distress and greater urges to acquire the item of interest than did CC participants. Nevertheless, subjective distress decreased in both groups following the acquiring task. There were no differences in acquiring-related distress between the CR and TL conditions. The findings indicate that subjective distress may decrease after relatively short periods of time in individuals with HD, but that CR may not alleviate acquiring-related distress in HD participants.
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