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

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)