Abstract

Low brain tissue perfusion due to abnormal venous drainage is thought to be a central mechanism of brain damage in SWS. Here, HR-PWI was used to quantify WM perfusion abnormalities and to correlate these with brain atrophy and clinical variables. Fourteen children (age range, 0.8-10.0 years) with unilateral SWS underwent MR imaging examinations, including HR-PWI. rCBV, rCBF, and MTT in the affected WM and in contralateral homotopic WM were measured. AI for each perfusion parameter was correlated with age, brain atrophy, and motor and seizure variables as well as IQ. Increased perfusion was seen in the affected hemisphere in 5 children and decreased perfusion in 9 children. Brain atrophy was more severe in the low-perfusion group (P = .01) and was related to both CBF-AI and CBV-AI (r = -0.69, P = .007; r = -0.64, P = .014, respectively). Older children had lower CBV values on the affected side (r = -0.62, P = .02). Longer duration of epilepsy was related to lower CBF (more negative CBF-AI, r = -0.58, P = .03) and low CBV (r = -0.55, P = .04) on the affected side. Lower perfusion was associated with more frequent seizures (rCBF-AI: r = -0.56, P = .04; rCBV-AI: r = -0.63, P = .02). Increased perfusion in the affected cerebral WM may indicate an early stage of SWS without severe brain atrophy. Decreased perfusion is associated with frequent seizures, long duration of epilepsy, and brain atrophy.

Highlights

  • AND PURPOSE: Low brain tissue perfusion due to abnormal venous drainage is thought to be a central mechanism of brain damage in SWS

  • Increased perfusion in the affected cerebral WM may indicate an early stage of SWS without severe brain atrophy

  • Intraclass and Interclass Reliability of Hemispheric Atrophy and Perfusion Measurements The intraclass and interclass reliability of rCBF, rCBV, and rMTT in the SWS cases and controls and BA-AI values in the SWS subjects were beyond our target value of 0.90, varying from 0.93 to 0.98 and from 0.94 to 0.97, respectively

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Summary

Methods

Fourteen children (age range, 0.8 –10.0 years) with unilateral SWS underwent MR imaging examinations, including HR-PWI. rCBV, rCBF, and MTT in the affected WM and in contralateral homotopic WM were measured. Fourteen children (age range, 0.8 –10.0 years) with unilateral SWS underwent MR imaging examinations, including HR-PWI. 10 healthy adults (5 men and 5 women; age range, 22– 47 years; median age, 34 years) were recruited as controls to establish normal perfusion parameter asymmetries between hemispheres. Exclusion criteria for this group included a history of a neurologic or psychiatric condition, head trauma with loss of consciousness for Ͼ5 minutes, a habit of drug or alcohol abuse, brain surgery, as well as focal hyperintensity in the brain on T2-weighted images. The study was approved by the Wayne State University Human Investigation Committee, and informed consent of the parent or legal guardian was obtained

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