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Table 2 Metabolite concentrations in basal ganglia and parieto-occipital white matter

From: MR spectroscopy-based brain metabolite profiling in propionic acidaemia: metabolic changes in the basal ganglia during acute decompensation and effect of liver transplantation

Basal ganglia metabolites (mM, mean (standard deviation))
  Propionic Acidaemia Normal MRI comparator group
  Metabolic stability (6 studies from 6 patients) Acute encephalopathy (5 studies from 3 patients) Post-liver transplant (2 studies from 2 patients) (63 studies)
Median age (years, (range)) 3.6 (0.6-15.9) 6.13 (1.0-9.4) 14.4 (12.9-15.9) 4.1 (0.5-16.7)
N-Acetylaspartate 5.03 (0.53) 3.89 (0.20)†# 5.35 (0.28) 5.21 (0.51)
Creatine 4.57 (0.47) 3.71 (0.58)† $ 4.42 (0.14) 4.55 (0.59)
Glutamine 1.29 (0.30) * 1.00 (1.00)* 1.27 (0.25) 1.98 (0.79)
Glutamate 4.28 (0.25) 3.07 (0.60)†# 4.30 (0.39) 4.45 (0.78)
Glutamine+Glutamate 5.57 (0.41) * 4.07 (1.19)† $ 5.57 (0.28) 6.43 (1.39)
myo-Inositol 2.58 (0.26) 2.79 (0.90) 2.39 (0.0) 2.49 (0.43)
Lactate 0.21 (0.25) 0.93 (0.34)†# 0.00 (0.32) 0.11 (0.20)
Choline 1.10 (0.18) 1.16 (0.29) 0.90 (0.63) 1.11 (0.18)
White matter metabolites (mM, mean (standard deviation))
  Propionic Acidaemia Normal MRI comparator group
  Metabolic stability (5 studies from 5 patients) Acute encephalopathy (4 studies from 3 patients) Post-liver transplant (2 studies from 2 patients) (53 studies)
Median age (years, (range)) 3.1 (0.6-15.9) 7.74 (3.0 -9.4) 14.4 (12.9-15.9) 4.7 (0.5-16.7)
N-Acetylaspartate 5.28 (0.80) 4.50 (0.47) 6.11 (0.39) 5.11 (0.73)
Creatine 3.55 (0.19) * 3.33 (0.53) 3.70 (0.00) 3.18 (0.47)
Glutamine 1.43 (0.54) 1.34 (1.30) 1.62 (0.49) 1.32 (0.62)
Glutamate 3.83 (0.45) 2.65 (0.44)*$ 4.27 (0.31) 3.51 (0.78)
Glutamine+Glutamate 5.26 (0.89) 3.98 (1.54) 5.89 (0.80) 4.84 (1.18)
myo-Inositol 2.73 (0.31) 2.57 (0.39) 2.79 (0.02) 2.57 (0.39)
Lactate 0.40 (0.30) 0.81 (0.74)* 0.32 (0.45) 0.21 (0.28)
Choline 0.93 (0.15)* 1.12 (0.37) 0.83 (0.09) 1.08 (0.15)
  1. Comparison between propionic acidaemia cohorts and normal MRI comparator group.
  2. Mann Whitney U test (2 tailed exact significance) for difference in metabolite concentrations between Normal MRI Comparator group and propionic acidaemia cohorts,*p < 0.05, † p < 0.01. Mann Whitney U test (2 tailed exact significance) for difference in metabolite concentrations between stable and encephalopathic propionic acidaemia cohort, $ p < 0.05, # p < 0.01
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