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Abstract #1123

Measurement of Disease Severity in Niemann-Pick Disease Type C Using 3T MRS

Eva H. Baker1, Nicole M. Yanjanin2, Andrea L. Gropman3, Forbes D. Porter2

1Diagnostic Radiology Department, Clinical Center, NIH, Bethesda, MD, USA; 2National Institute of Child Health and Development, NIH, Bethesda, MD, USA; 3Children's National Medical Center, Washington, DC, USA

This paper is part of an ongoing longitudinal study of patients with NPC that includes quantitative measurements of metabolite levels at four locations in the brain. In a multivariate analysis, correlations were found between NAA and disease severity at 4 out of 4 locations studied, and between mI and disease severity at 1 location. NAA and mI also varied with age at some locations.