Brain proton MR spectroscopy measurements in CLN3 disease.

An N Dang Do,Eva H Baker,Cristan A Farmer,Ariane G Soldatos,Audrey E Thurm,Forbes D Porter

Molecular genetics and metabolism（2023）

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摘要

Based on their correlations to established assessments, NAA and glutamine/glutamate/GABA measured in the midline parietal gray matter may be useful indicators of CLN3 disease state. In a clinical trial, divergence of the MRS measurements and clinical severity markers from age may be useful as surrogate measures for treatment responses.

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关键词

Glx,Juvenile neuronal ceroid lipofuscinosis,NAA,Neurodevelopment

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