Age | Treatment | Glycine plasma | Glycine | Creatine | PhCholine | NAA | mI + Gly | Gly/Crea | Gly + mI/crea | Gly/crea |
---|---|---|---|---|---|---|---|---|---|---|
Days | Value (SD%) | Value (SD%) | Value (SD%) | Value (SD%) | Value (SD%) | Height ratio 35Â ms | Height ratio 144Â ms | |||
Basal ganglia | ||||||||||
Patient 1 | ||||||||||
 5 | None | 820 | 7.55 (9%) | 7.87 (4%) | 2.45 (5%) | 5.42 (7%) | 12.21 (14%) | 0.96 | 0.86 | 0.76 |
 17 | Benzoate | 147 | 5.17 (16%) | 8.31 (5%) | 2.99 (6%) | 6.96 (7%) | 12.23 (20%) | 0.62 | 0.61 | 0.55 |
 181 | Benzoate | 352 | 5.02 (12%) | 12.04 (4%) | 3.63 (5%) | 11.72 (4%) | 5.02 (12%) | 0.42 | 0.34 | 0.27 |
 230 | KD + LDB | 237 | 5.64 (12%) | 11.48 (4%) | 3.17 (5%) | 11.32 (5%) | 5.64 (12%) | 0.48 | 0.39 | 0.33 |
 413 | KD + LDB | 231 | NA | NA | NA | NA | NA | 0.25 | 0.36 | 0.27 |
Patient 2 | ||||||||||
 4 | None | 2108 | NA | NA | NA | NA | NA | NA | 0.87 | 0.98 |
 18 | Benzoate | 403 | 8.04 (7%) | 9.85 (4%) | 1.72 (5%) | 5.81 (6%) | 8.04 (7%) | 0.82 | 0.65 | 0.59 |
 36 | KD + LDB | 263 | 8.23 (10%) | 7.91 (6%) | 2.78 (7%) | 5.62 (9%) | 9.60 (25%) | 1.04 | 0.77 | 0.69 |
Patient 3 | ||||||||||
 52 | Benzoate | 281 | NA | NA | NA | NA | NA | NA | 0.21 | NA |
 498 | KD + LDB | 190 | NA | NA | NA | NA | NA | NA | 0.16 | NA |
*Control neonates (N = 9) | ||||||||||
AVG range* | N/A |  | 1.23 (0.0–3.10) | 7.18 (3.77–11.4) | 3.32 (2.25–4.42) | 5.29 (3.46–7.83) | 6.84 (4.31–9.81) | 0.18 (0.0–0.31) | 0.47 (0.36–0.59) | 0.31 (0.21–0.38) |
White matter | ||||||||||
Patient 1 | ||||||||||
 5 | None | 860 | 2.95 (18%) | 3.22 (7%) | 1.73 (5%) | 2.54 (10%) | 8.72 (17%) | 0.91 | NA | 0.91 |
 17 | Benzoate | 147 | 1.63 (64%) | 5.00 (10%) | 2.50 (8%) | 4.60 (13%) | 8.18 (35%) | 0.33 | NA | 0.45 |
 181 | Benzoate | 352 | 2.89 (22%) | 7.46 (5%) | 2.65 (5% | 8.91 (4%) | 4.49 (43%) | 0.39 | NA | 0.28 |
 230 | KD + LDB | 237 | 3.02 (15%) | 6.84 (5%) | 2.47 (5%) | 8.46 (4%) | 3.02 (15%) | 0.44 | NA | 0.31 |
 413 | KD + LDB | 231 | NA | NA | NA | NA | NA | 0.49 | NA | 0.37 |
Patient 2 | ||||||||||
 4 | None | 2108 | NA | NA | NA | NA | NA | NA |  | 1.26 |
 18 | Benzoate | 403 | 1.14 (45%) | 3.51 (7%) | 1.72 (5%) | 2.65 (9%) | 8.12 (17%) | 0.23 | NA | 0.59 |
 36 | KD + LDB | 263 | 3.16 (17%) | 3.10 (10%) | 1.44 (8%) | 2.96 (12%) | 3.16 (17%) | 1.02 | NA | 0.77 |