Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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December 24, 2007
Sturge Weber Syndrome
- Features: pial angiomatosis (85% in one hemisphere), impaired cortical venous drainage, deep venous collaterals (including choroid plexus whose size correlates with extent of pial angioma)
- Cortical calcifications may be due to venous stasis and subsequent ischemia. Once cortex is damaged, the abnormal deep veins remain and abnormal white matter extends beyond cortical lesions, which may explain significant cognitive impairment in some patients.
- On perfusion SW patients show increased MTT, delayed TTP and decreased rCBF. rCBV may be slightly elevated (due to blood pooling in deep veins) or highly elevated in face of recent seizures like the patient here shown.
- Genetic defect is not well established although some patients have shown inversion of an arm of 4q and trisomy 10.
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