Abstract #3772
Assessment of metabolic abnormalities from the cerebellar region of the brain of a canine model of mucopolysaccharidosis type I (MPS I) using in vivo 1H MRS
Manoj Kumar 1 , Jessica Bagel 2 , Patricia ODonnell 3 , Katherine P Ponder 4 , James M Wilson 5 , Mark Haskins 3 , Charles H Vite 2 , and Harish Poptani 1
1
Radiology, University of Pennsylvania,
Philadelphia, PA, United States,
2
Clinical
Studies, University of Pennsylvania, Philadelphia, PA,
United States,
3
Pathobiology, University of
Pennsylvania, Philadelphia, PA, United States,
4
Internal
Medicine, Washington University School of Medicine, St.
Louis MO, United States,
5
Pathology
and Laboratory Medicine, University of Pennsylvania,
Philadelphia, PA, United States
In vivo 1H MRS was performed on MPS I (n=8) and normal
dogs (n=4) from the cerebellar regions of the brain to
assess metabolic changes. LC-model was used to measure
concentration of the major brain metabolites [N-acetylaspartate
(NAA), creatine (Cr), choline (Cho), and myo-inositol (mI)].
NAA, Cho, and mI peaks values were normalized to Cr peak
values. We observed significantly reduced NAA/Cr and
significantly increased Cho/Cr in MPS I along with
slightly elevated mI/Cr reflecting increased
demyelination, gliosis, and axonal injury. Our studies
indicate that 1H MRS might be a sensitive technique for
detecting and quantifying the neuroaxonal injury status
in this model.
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