Table 1.
Demographic, clinical, and paraclinical data of the Danish narcolepsy population by presence of cataplexy and hypocretin deficiency
| Narcolepsy with cataplexy | Narcolepsy without cataplexy | P-value | Low CSF hcrt-1 | Normal CSF hcrt-1 | P-value | |
|---|---|---|---|---|---|---|
| N = 46 | N = 14 | N = 43 | N = 17 | |||
| Demography | ||||||
| Gender (male), n (%) | 20 (43.5%) | 7 (50.0%) | 0.76 | 19 (44.2%) | 8 (47.1%) | 1.00 |
| Age (y), mean ± SEM | 36.18 ± 2.37 | 29.57 ± 2.96 | 0.18 | 36.2 ± 2.56 | 30.71 ± 2.32 | 0.28 |
| Age at disease onset (y), mean ± SEM | 18.80 ± 1.17 | 15.96 ± 1.73 | 0.19 | 18.17 ± 1.21 | 18.06 ± 1.76 | 0.99 |
| Disease duration (y), mean ± SEM | 17.37 ± 2.19 | 13.61 ± 2.76 | 0.63 | 18.01 ± 2.29 | 12.65 ± 2.42 | 0.32 |
| HLA-DQB1*0602-positivity,n (%) | 39 (95.12%) | 4 (30.77%) | < 0.01 | 38 (97.4%) | 5 (33.3%) | < 0.01 |
| N = 41 | N = 13 | N = 39 | N = 15 | |||
| CSF hcrt-1 < 129 pg/mL, n (%) | 40 (87.0%) | 3 (21.4%) | < 0.01 | - | - | |
| N = 46 | N = 14 | |||||
| Sleepiness | ||||||
| Daytime sleepiness, n (%) | 46 (100%) | 14 (100%) | 1.00 | 43 (100%) | 17 (100%) | 1.00 |
| Epworth Sleepiness Scale, (sum), mean ± SEM | 18.71 ± 0.50 | 16.50 ± 0.86 | 0.03 | 18.42 ± 0.55 | 17.56 ± 0.72 | 0.29 |
| N = 45 | N = 14 | N = 43 | N = 16 | |||
| Age at onset (y), mean ± SEM | 19.32 ± 1.34 | 15.96 ± 1.73 | 0.23 | 18.49 ± 1.43 | 18.65 ± 1.58 | 0.67 |
| N = 46 | N = 14 | N = 43 | N = 17 | |||
| Cataplexy characteristics* | ||||||
| Frequency** | ||||||
| Grade 1, n (%) | 1 (2.2%) | 0 (0%) | - | 0 (0%) | 1 (16.7%) | |
| Grade 2, n (%) | 4 (8.7%) | 0 (0%) | 3 (7.5%) | 1 (16.7%) | 0.036 | |
| Grade 3, n (%) | 4 (8.7%) | 0 (0%) | 3 (7.5%) | 1 (16.7%) | ||
| Grade 4, n (%) | 17 (37.0%) | 0 (0%) | 17 (42.5%) | 0 (0%) | ||
| Grade 5, n (%) | 20 (43.5%) | 0 (0%) | 17 (42.5%) | 3 (50.0%) | ||
| Injurious cataplexy, n (%) | 21 (47.7%) | 0 (0%) | - | 18 (46.2%) | 3 (60.0%) | 0.66 |
| N = 44 | N = 39 | N = 5 | ||||
| Age at onset (y), mean ± SEM | 22.16 ± 1.45 | - | - | 22.56 ± 1.56 | 19.50 ± 4.02 | 0.61 |
| N = 46 | N = 0 | N = 40 | N = 6 | |||
| Hypnagogic Hallucinations | ||||||
| Hypnagogic hallucinations, n (%) | 41 (89.1%) | 7 (50.0%) | < 0.01 | 37 (86.0%) | 11 (64.7%) | 0.08 |
| Age at onset (y), mean ± SEM | 21.70 ± 1.45 | 16.71 ± 1.87 | 0.14 | 21.94 ± 1.53 | 17.72 ± 2.10 | 0.23 |
| N = 40 | N = 7 | N = 36 | N = 11 | |||
| Sleep paralysis | ||||||
| Sleep paralysis, n (%) | 35 (76.1%) | 4 (28.6%) | < 0.01 | 31 (72.1%) | 8 (47.1%) | 0.08 |
| Age at onset (y), mean ± SEM | 22.91 ± 1.60 | 12.33 ± 3.48 | 0.05 | 23.45 ± 1.69 | 16.0 ± 3.37 | 0.10 |
| N = 35 | N = 3 | N = 31 | N = 7 | |||
| Automatic Behaviour | ||||||
| Automatic behaviour, n (%) | 25 (61.0%) | 7 (53.9%) | 0.64 | 22 (55.0%) | 10 (71.4%) | 0.35 |
| N = 41 | N = 13 | N = 40 | N = 14 | |||
| Disrupted night sleep*** | ||||||
| Awakenings/ night, mean ± SEM | 5.60 ± 0.60 | 2.93 ± 0.94 | 0.01 | 5.78 ± 0.61 | 3.00 ± 0.89 | < 0.01 |
| N = 43 | N = 14 | N = 40 | N = 17 | |||
| MSLT (multiple sleep latency test) | ||||||
| Sleep latency (seconds) mean ± SEM | 228.82 ± 23.97 | 267.14 ± 38.20 | 0.20 | 214.10 ± 22.29 | 297.59 ± 42 | 0.048 |
| N = 46 | N = 14 | N = 43 | N = 17 | |||
| SOREMPs (number) mean ± SEM | 3.04 ± 0.18 | 3.29 ± 0.24 | 0.74 | 3.33 ± 0.16 | 2.53 ± 0.31 | 0.02 |
| N = 46 | N = 14 | N = 43 | N = 17 |
*
Comparisons within the cataplexy group.
**
1-5 indicates cataplexy attacks 1/year or less, 1/month, 1/week, several times/week, or 1 or several/day.
***
Number of awakenings on polysomnography. SOREMPs (sleep onset REM periods), CSF hcrt-1 (hypocretin-1 levels in the cerebrospinal fluid).