Table S1.
Narcolepsy with cataplexy by puberty status at both onset and diagnosis
| Diagnosis/Onset Prepuberty | Diagnostic-Puberty Onset-Prepuberty | Diagnosis/Onset Puberty | Diagnostic-Postpuberty Onset-Prepubery | Diagnostic-Postpuberty Onset-Puberty | Diagnosis/Onset Postpuberty | |
|---|---|---|---|---|---|---|
| n = 142 | n = 42 | n = 35 | n = 23 | n = 24 | n = 5 | |
| Age (y) | 7.56 ± 0.12† | 12.12 ± 0.22§,††† | 12.40 ± 0.24††† | 15.39 ± 0.29†† | 15.71 ± 0.29 | 16.80 ± 0.63 |
| 70.21% | 66.67% | 60.00% | 60.87% | 66.67% | 100.00% | |
| Sex | 19.61 ± 0.42 (139)† | 22.80 ± 0.77 (41)§§ | 22.75 ± 0.85 (34)§§ | 27.59 ± 1.03 | 25.22 ± 1.01 | 21.89 ± 2.21 |
| BMI (kg/m2) | 6.72 ± 0.14† | 7.76 ± 0.25* | 11.43 ± 0.28††† | 8.04 ± 0.34** | 12.58 ± 0.34†† | 15.20 ± 0.73 |
| Age of disease onset (sleepiness or cataplexy) (y) | 6.78 ± 0.13 (140)† | 8.00 ± 0.25* | 11.46 ± 0.27††† | 8.26 ± 0.33** | 12.79 ± 0.33†† | 15.20 ± 0.71 |
| Age of onset (EDS) (y) | 6.92 ± 0.14 (140)† | 8.38 ± 0.26 (40)* | 11.49 ± 0.28††† | 9.38 ± 0.36 (21)** | 13.00 ± 0.34†† | 15.40 ± 0.74 |
| Age of onset (cataplexy) (y) | 58.39% (137) | 38.10% | 48.49% (33) | 56.52% | 79.17% | 60.00% |
| % Hypnagogic hallucinations | 6.80 ± 0.18 (79)† | 7.13 ± 0.40 (15)&&&,* | 11.56 ± 0.39 (16)** | 10.46 ± 0.43 (13)** | 12.95 ± 0.36 (19)†† | 15.00 ± 0.90 (3) |
| Age of onset (hallucinations) (y) | 14.71% (136) | 15% (40) | 40.63% (32) | 43.48% | 66.67%†† | 60.00% |
| % Sleep paralysis | 7.17 ± 0.39 (18)† | 8.83 ± 0.68 (6)* | 11.58 ± 0.48 (12)&&&,** | 9.90 ± 0.52 (10)** | 13.06 ± 0.41 (16)†† | 15.00 ± 0.96 (3) |
| Age of onset (sleep paralysis) (y) | 96.26% (107) | 86.21% (29) | 93.75% (16) | 87.50% (16) | 80.00% (20) | 100% (4) |
| % Disturbed nocturnal sleep | 14.29% (91) | 7.14% (28) | 8.33% (24) | 0% (19) | 11.11% (18) | 25.00% (4) |
| MSLT sleep latency (min) | 3.24 ± 0.19 (138) | 2.67 ± 0.34 | 2.60 ± 0.39 (23) | 3.37 ± 0.47 | 4.28 ± 0.471 (23) | 5.47 ± 1.00 |
| MSLT SOREMP numbers | 4.48 ± 0.08 (137) | 4.57 ± 0.14 | 4.00 ± 0.16 (33) | 4.39 ± 0.19 | 4.25 ± 0.18 | 3.80 ± 0.40 |
| MSLT SOREMP (mean latency) | 2.09 ± 0.13 (136) | 1.98 ± 0.23 | 2.91 ± 0.26 (33) | 2.60 ± 0.32 | 3.23 ± 0.31 | 3.87 ± 0.68 |
| MSLT % < 8 and ≥ 2 SOREMP | 96.35% (137) | 95.24% | 93.75 % (32) | 91.30% | 95.65% (23) | 80.00% |
| AHI | 10.8 ± 0.18 (104) | 1.10 ± 0.32 (33)§ | 1.40 ± 0.38 (24) | 0.60 ± 0.40 (22) | 0.77 ± 0.39 (23) | 1.00 ± 0.83 |
| % AHI > 10 | 0.96% (104) | 3.03% (33) | 4.17% (24) | 0% (22) | 0% (23) | 0% |
| % Hypocretin-1 ≤ 110 | 85.71% (7) | 87.5% (8) | 100% (6) | 100% (8) | 100% (5) | 50% (2) |
| % Hypocretin-1 ≤ 200 | 100% (7) | 87.5% (8) | 100% (6) | 100% (8) | 100% (5) | 50% (2) |
| DQB1*0602 | 94.66% (131) | 96% (50) | 94.83% (58) | 100% (17) | 100% (20) | 90% (10) |
| % ASO ≥ 100 | 25.86% (58) | 8.33% (12) | 46.15% (13) | 50% (10) | 21.05% (19) | 33.33% (3) |
| % ASO ≥ 200 | 20.69% (58) | 8.33% (12) | 46.15% (13) | 20% (10) | 5.26% (19) | 33.33% (3) |
| % ADB ≥ 480 | 12.07% (58) | 0% (12) | 15.39% (13) | 20% (10) | 0% (19) | 0% (3) |
| Disease duration (blood sample-onset) (y) | 0.85 ± 0.13 (135)† | 4.39 ± 0.24 (41)***,& | 0.97 ± 0.26§§ | 7.30 ± 0.32** | 3.13 ± 0.31†† | 1.60 ± 0.69 |
Unadjusted comparisons:
†
P < 0.001 vs other groups.
§
P < 0.05 vs group 3;
†††
P < 0.001 vs group 4, 5, 6;
††
P < 0.01 versus group 6;
§§
P < 0.01 versus group 4, 5;
&
P < 0.05 versus group 5;
*
P < 0.001 versus group 1, 3, 5, 6;
&&&
P < 0.05 versus group 4;
***
P < 0.001 versus group 3, 4, 6;
**
P < 0.001 versus group 5, 6.