Clinical and metabolic correlates of cerebral calcifications in Sturge-Weber syndrome.
Vinod K Pilli, Michael E Behen, Jiani Hu and 4 others
PMID 28397986WHAT IT FOUND
Brain calcification volume in Sturge-Weber syndrome increased over time and correlated with lower IQ scores.
Larger calcification was also linked to younger age at seizure onset, suggesting it may serve as an imaging marker for cognitive outcome in young children.
Key findings
01Larger calcified brain volume at baseline was associated with longer duration of epilepsy, younger age at seizure onset, and lower IQ measured at follow-up.
02Mean calcified brain volume increased significantly from baseline to follow-up, with the rate of increase being faster in children with younger age at epilepsy onset.
03All patients showed abnormal glucose metabolism in the affected hemisphere, and all calcified lobes showed hypometabolism, which often extended beyond the calcified areas.
STILL TO COME
How it was doneWhat they foundWhat it means for PTsWhat it means for OTsWhat it means for SLPs
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What it does not show
The study population was small (15 children) due to the rarity of Sturge-Weber syndrome. The association between baseline calcification volume and IQ showed only a trend after correction for multiple comparisons. Calcification volumes were measured manually by a single investigator, which may introduce inaccuracies despite blinding. MRI scans were performed on two different scanner types, though the authors state this did not significantly affect results.
The easy way to misread this
Do not assume calcification volume is a definitive independent predictor of cognitive outcome. The correlation with IQ was not statistically significant after correcting for multiple testing, and the study was too small to confirm this as a reliable clinical marker.