The correlation between motor impairment and cerebral blood flow in Sturge-Weber syndrome
Journal
European Journal of Paediatric Neurology
Journal Volume
11
Journal Issue
2
Pages
96-103
Date Issued
2007
Author(s)
Abstract
Recent studies of regional cerebral blood flow (rCBF) and cerebral metabolism in patients with Sturge-Weber syndrome (SWS) have demonstrated a regional decrease of CBF and metabolism in the affected hemisphere, which may reflect the neurological status of the patients. Therefore, in the present study, we use stable xenon computed tomography (xenon-CT) and/or 99mtechnetium hexamethylprophylene amine oxime (99mTcHMPAO) single photon emission computed tomography (SPECT) to determine whether the degree of cerebral hemodynamic disturbance correlates with the severity of motor impairment in 5 children with Sturge-Weber syndrome. We found that 5 patients had great variation in clinical progression and outcome, but MR imaging investigations all revealed extensive but stable abnormalities. rCBF, assessed by stable xenon-CT and/or 99mTcHMPAO-SPECT, showed perfusion defect in all subjects. However, the xenon-CT disclosed reduction of rCBF and cerebrovascular reactivity in patients with severe motor paralysis. The total CBF and rCBF detected by xenon-CT were improved after clinical status was stabilized. We conclude that the motor function impairment in patients with SWS appeared to better correlate with the degree of rCBF impairment than with the abnormalities in conventional neuroimaging. Therefore, the quantitative CBF study is one of the diagnostic modality of choice in evaluating the neurological status in patients with SWS. ? 2006.
SDGs
Other Subjects
anticoagulant agent; anticonvulsive agent; hexamethylpropylene amine oxime technetium tc 99m; xenon; angioma; article; blood vessel reactivity; brain blood flow; brain blood vessel; brain metabolism; brain perfusion; brain region; child; clinical article; computer assisted tomography; correlation analysis; diagnostic value; disease course; disease severity; electroencephalography; female; hemisphere; human; male; medical assessment; medical decision making; motor dysfunction; neuroimaging; nuclear magnetic resonance imaging; outcome assessment; paralysis; priority journal; quantitative analysis; seizure; single photon emission computer tomography; Sturge Weber syndrome; Blood Circulation Time; Brain Mapping; Cerebrovascular Circulation; Child; Child, Preschool; Electroencephalography; Female; Humans; Magnetic Resonance Imaging; Male; Movement Disorders; Organotechnetium Compounds; Regional Blood Flow; Statistics; Sturge-Weber Syndrome; Tomography, Emission-Computed, Single-Photon
Type
journal article