Table 2. Reference Ranges for CSF Concentrations of Neurotransmitter Metabolites and Pterinsa.
| mean
(med.) |
|||||||
|---|---|---|---|---|---|---|---|
| groups | 5-HIAA | HVA | HVA/5-HIAA | 3-OMD | MHPG | NH2 | BH2 |
| A (n = 27) | 661 (620) | 868 (838) | 1.31 (1.37) | 109 (100) | 62 (56) | 19 (17) | 27 (26) |
| 0–1 month | 426–1186 | 405–1430 | 0.81–1.73 | 37–156 | 37–110 | 7–36 | 9–59 |
| A vs B | p = 0.002 | p = 0.111 | p = 0.122 | p < 0.001 | p = 0.172 | p = 0.543 | p = 0.030 |
| B (n = 166) | 468 (441) | 726 (700) | 2 (2) | 74 (61) | 54 (47) | 18 (15) | 21 (18) |
| 1 month to 0.5 year | 185–990 | 274–1487 | 0.8–3.7 | 11–250 | 18–138 | 7–65 | 6–69 |
| B vs C | p < 0.001 | p < 0.001 | p < 0.001 | p < 0.001 | p < 0.001 | p = 0.704 | p < 0.001 |
| C (n = 457) | 249 (235) | 555 (539) | 2.33 (2.23) | 32 (26) | 35 (31) | 19 (16) | 16 (14) |
| 0.5–3 years | 111–631 | 174–1176 | 0.79–4.95 | 4–209 | 11–106 | 5–50 | 5–51 |
| C vs D | p < 0.001 | p < 0.001 | p < 0.001 | p < 0.001 | p < 0.001 | p = 0.667 | p = 0.758 |
| D (n = 328) | 179 (170) | 473 (463) | 2.78 (2.71) | 21 (15) | 30 (26) | 19 (17) | 16 (113) |
| 3–7 years | 46–583 | 62–1185 | 1.29–5.96 | 3–179 | 8–67 | 6–69 | 5–48 |
| D vs E | p < 0.001 | p < 0.001 | p = 0.159 | p = 0.490 | p = 0.490 | p = 0.498 | p = 0.666 |
| E (n = 222) | 156 (149) | 402 (386) | 2.69 (2.63) | 20 (13) | 30 (27) | 19 (17) | 16 (14) |
| 7–11 years | 57–406 | 81–815 | 1.20–4.61 | 4–113 | 14–66 | 6–40 | 6–55 |
| E vs F | p < 0.001 | p < 0.001 | p < 0.001 | p = 0.069 | p = 0.003 | p = 0.669 | p = 0.009 |
| F (n = 186) | 132 (129) | 309 (300) | 2.41 (2.34) | 17 (12) | 27 (10) | 19 (17) | 14 (12) |
| 11–16 years | 46–276 | 67–669 | 0.85–5.74 | 3–69 | 12–71 | 6–47 | 5–41 |
| F vs G | p < 0.001 | p < 0.001 | p < 0.001 | p = 0.656 | p = 0.913 | p = 0.100 | p = 0.421 |
| G (n = 130) | 113 (112) | 241 (215) | 2.20 (2.07) | 18 (12) | 27 (25) | 21 (20) | 14 (11) |
| over 16 years | 40–186 | 65–488 | 1.04–4.90 | 3–55 | 12–59 | 7–51 | 5–45 |
| Known Metabolic Disorders (n = 18) and Elevated Levels of NH2 (n = 115) | |||||||
| Segawa (n = 2) 4.2 and 5.6 years | 10–17 | 15–20 | 1.50–1.18 | 2–3 | 4–6 | 2–4 | 1–3 |
| SR (n = 1) 27.88 years | 21 | 56 | 0.72 | 25 | 3 | 35 | 67 |
| TH (n = 11) 0.01–18.63 years | 212 (189) | 129 (117) | 0.62 (0.62) | 40 (22) | 35 (33) | 15 (12) | 28 (26) |
| 104–405 | 44–256 | 0.31–1.1 | 5–167 | 7–71 | 8–49 | 6–58 | |
| AADCb (n = 3) 0.46–2.40 years | 9 (3) | 15 (15) | 5.55 (7.50) | 1939 (2500) | 28 (25) | 15 (17) | 21 (21) |
| 2–23 | 3–27 | 0.15–9 | 252–3065 | 7–50 | 10–55 | 14–47 | |
| PTPS (n = 1) 24.46 years | 15 | 109 | 7.38 | 29 | 11 | 4 | 5 |
| DTDS (n = 1) 0.92 year | 302 | 2426 | 8.03 | 96 | 38 | 48 | 7 |
| elevated levels of NH2 (n = 115) | 258 (232) | 498 (490) | 2.09 (2.11) | 44 (29) | 34 (28) | 316 (184) | 38 (24) |
| 0.02–60.64 years | 26–732 | 23–1091 | 0.49–3.84 | 3–116 | 4–76 | 90–2500 | 21–91 |
a
Results are expressed in nanometers as average (median) and range.
b
For AADC, the main concentration of 5-HTrp is 505 nM, with a median of 316 nM (range: 300–900 nM).