PROTON MAGNETIC-RESONANCE SPECTROSCOPY IN CHILDREN WITH STURGE-WEBER-SYNDROME

Quantitative proton magnetic resonance spectroscopy was performed on s ix children with Sturge-Weber syndrome following gadolinium enhanced m agnetic resonance imaging (MRI). MRI revealed only unilateral involvem ent in all cases. The mean concentration (mmol/kg wet weight) of the n euronal marker N-acetyl-aspartate was significantly reduced by 37% in the ipsilateral gadolinium enhanced volume of interest compared to a s imilarly placed contralateral volume of interest (5.39 +/- 1.70 [SD] v s 8.50 +/- 1.14, P <.005, two-tailed paired Student's t-test), Decreas ed N-acetyl-aspartate in the ipsilateral volume of interest was observ ed in all patients studied. No significant differences were found in t he concentrations of creatine/phosphocreatine or choline compounds bet ween the ipsilateral and contralateral volumes of interest. These find ings give possible new insight into the pathophysiology of this diseas e and suggest that quantitative proton magnetic resonance spectroscopy may be useful for the early characterization and monitoring of neuron al dysfunction or loss in infants and children with Sturge-mieber synd rome.