Key result
In children with Sturge-Weber syndrome, N-acetyl-aspartate concentration was reduced by 37% in the affected hemisphere compared to the unaffected hemisphere (5.39 vs 8.50 mmol/kg; P<.005).
Why the study?
Does quantitative proton magnetic resonance spectroscopy detect neuronal dysfunction in children with Sturge-Weber syndrome?
Observational (n=6)
Does quantitative proton magnetic resonance spectroscopy detect neuronal dysfunction in children with Sturge-Weber syndrome?
Absolute Event Rate: 5.39% vs 8.5%
p-value: p=<.005
Quantitative proton magnetic resonance spectroscopy demonstrates reduced N-acetyl-aspartate in affected brain regions of children with Sturge-Weber syndrome, suggesting its utility for monitoring neuronal dysfunction.
Supports MRS as biomarker of neuronal integrity in Sturge-Weber syndrome; leaves open clinical utility pending validation.
Quantitative proton magnetic resonance spectroscopy was performed on six children with Sturge-Weber syndrome following gadolinium enhanced magnetic resonance imaging (MRI). MRI revealed only unilateral involvement in all cases. The mean concentration (mmol/kg wet weight) of the neuronal marker N-acetyl-aspartate was significantly reduced by 37% in the ipsilateral gadolinium enhanced volume of interest compared to a similarly placed contralateral volume of interest (5.39 +/- 1.70 [SD] vs 8.50 +/- 1.14, P < .005, two-tailed paired Student's t-test). Decreased N-acetyl-aspartate in the ipsilateral volume of interest was observed in all patients studied. No significant differences were found in the concentrations of creatine/phosphocreatine or choline compounds between the ipsilateral and contralateral volumes of interest. These findings give possible new insight into the pathophysiology of this disease and suggest that quantitative proton magnetic resonance spectroscopy may be useful for the early characterization and monitoring of neuronal dysfunction or loss in infants and children with Sturge-Weber syndrome.
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Moore et al. (1998) conducted an observational in Sturge-Weber syndrome (n=6). Affected hemisphere (ipsilateral gadolinium enhanced volume of interest) vs. Unaffected hemisphere (contralateral volume of interest) was evaluated on Mean concentration of N-acetyl-aspartate (mmol/kg wet weight) (p=<.005). In children with Sturge-Weber syndrome, N-acetyl-aspartate concentration was reduced by 37% in the affected hemisphere compared to the unaffected hemisphere (5.39 vs 8.50 mmol/kg; P<.005).
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