Table 1. Narcolepsy patient demographics and results of antibodies to neuronal antigens.
Patient | Sex, age (y), time since onset (m) | CSF orexin A, MCH (pg/ml) | NMDAR (1:20) | CASPR2 (1:100) | LGI1 (1:20) | Contactin2 (1:100) | Dopamine D2 (1:20) | GABAA3 (1:20) | Immuno-fluorescence Tissue sections | Immuno-fluorescence Live hippocampal neurons |
---|---|---|---|---|---|---|---|---|---|---|
1 | F,16, 13 | <10, n.d. | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
2 | F,15, 13 | <10, 133 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
3 | M, 4,16 | <10, n.d. | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
4 | M, 9, 10 | 52, n.d. | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
5 | F, 8, 7 | <10, n.d. | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
6 | M, 11, 14 | <10, 141 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
7 | M, 12, 12 | <10, 263 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
8 | M, 16, 7 | <10, 217 | 0 | 0 | 0 | 0 | 0 | 0 | 2 | 0 |
9 | M, 14, 16 | <10, n.d. | 0 | 0 | 0 | 0 | 0 | 0 | 2 | 0 |
10 | M, 18, 13 | <10, 246 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
11* | M, 24, 41 | <10, 198 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
12 | F, 18, 20 | <10, n.d. | 0 | 1 | 0 | 0 | 0 | 0 | 2 | 0 |
13 | M, 17, 19 | 55, 298 | 0 | 0 | 0 | 0 | 0 | 0 | 1 | 0 |
All narcolepsy patients (n = 13) | Orexin <110 pg/ml (n = 13), MCH 213+/- 57 pg/ml (n = 8) | 0/13 | 1/13 | 0/13 | 0/13 | 0/13 | 0/13 | 4/13 | 0/13 | |
Orexin normal paediatric sera/CSFs (n = 15) | Orexin >200 pg/ml (n = 15), MCH 156+/- 38 pg/ml (n = 6) | 0/15 | 0/15 | 0/15 | 0/15 | 0/15 | 0/15 | 0/15 | 0/15 |
CSF orexin levels <110 pg/ml supports a diagnosis of type 1 narcolepsy (narcolepsy with cataplexy). MCH levels were measured in CSFs the CSFs that were still available and in one additional narcolepsy CSF (without a paired serum). Serum cell-based assays for antibodies to individual antigens, immunofluorescence for binding to orexin neurons on rodent brain tissue sections or to hippocampal neuronal cultures were scored subjectively by comparison with positive and negative controls: 0 = no binding, 1 = weakly positive, 2 = moderately positive, 3 = strongly positive, 4 = complete co-localisation.
*In patient 11 the onset of narcolepsy was 18 months before Pandemrix vaccination but his cataplexy worsened after vaccination. In all other cases the onset of symptoms was after Pandemrix vaccination.