Clinical features of patients with pan-neurofascin (panNF) antibodies and comparison with patients with neurofascin-155 (NF155), CNTN1, CNTN1/Caspr1 antibodies, and seronegative cohorts
| Clinical feature (n, %) | PanNF (n=8) | NF155 (n=15) | CNTN1 (n=11) | CNTN1/Caspr1 (n=8) | Seronegative (n=194) | OR vs NF155 | 95% CI | OR vs seronegative | 95% CI |
| Initial clinical diagnosis of GBS | 5/8 (63%) | 3/15 (20%) | 3/11 (27%) | 4/7 (57%) | 38/185 (21%) | 6.7 | 1.1 to 34.5 | 6.5 | 1.6 to 24.9 |
| Acute/subacute progression | 8/8 (100%) | 7/15 (47%) | 4/11 (36%) | 5/7 (71%) | 56/184 (30%) | ∞ | 2.0 to ∞ | ∞ | 4.8 to ∞ |
| Ataxia | 3/8 (38%) | 7/15 (47%) | 7/11 (64%) | 5/7 (71%) | 62/158 (39%) | 0.7 | 0.1 to 3.4 | 0.9 | 0.2 to 3.7 |
| Tremor | 0/8 (0%) | 5/15 (33%) | 3/11 (27%) | 3/7 (43%) | 39/154 (25%) | 0 | 0 to 1.3 | 0 | 0 to 1.4 |
| Neuropathic pain | 4/8 (50%) | 1/15 (7%) | 7/11 (64%) | 5/7 (71%) | 49/134 (37%) | 14 | 1.3 to 180 | 1.7 | 0.5 to 6.2 |
| Cranial nerve palsy | 8/8 (100%) | 5/15 (33%) | 5/11 (45%) | 1/7 (14%) | 41/156 (26%) | ∞ | 3.3 to ∞ | ∞ | 5.7 to ∞ |
| Autonomic dysfunction | 6/8 (75%) | 0/15 (0%) | 2/11 (18%) | 0/2 | 9/71 (13%) | ∞ | 6.1 to ∞ | 20.7 | 3.9 to 105.4 |
| Respiratory involvement | 7/8 (88%) | 0/15 (0%) | 3/11 (27%) | 0/7 | 25/185 (14%) | ∞ | 9.9 to ∞ | 44.8 | 7.3 to 506.3 |
| Nephrotic syndrome | 3/8 (38%) | 0/15 (0%) | 9/11 (82%) | 0/7 | 5/147 (3%) | ∞ | 1.9 to ∞ | 17 | 3.5 to 73.7 |
| MRI plexus/root abnormalities | 2/4 (50%) | 2/7 (29%) | 2/7 (29%) | 5/6 (83%) | 15/53 (28%) | 6.7 | 1.1 to 34.5 | 6.5 | 1.6 to 24.9 |
| Nadir mRS >4 | 8/8 (100%) | 3/15 (20%) | 4/11 (36%) | 2/7 (29%) | 38/185 (21%) | ||||
| Significant vs NF155 | P value | Significant vs seronegative | P value | ||||||
| Nadir mRS (median, range) | 5.5 (5–6) | 4 (2–5) | 4 (2–6) | 4 (4–5) | 3 (1–5) | ** | 0.006 | *** | <0.001 |
| CSF protein (g/L) (median, range) | 0.48 (0.34–0.62) | 1.65 (0.61–7.05) | 2 (0.24–5.9) | 2.7 (0.91–4.46) | 0.87 (0.18–6.0) | *** | <0.001 | * | 0.04 |
The 95% CI of the OR was calculated by the Baptista-Pike method. Nadir mRS and CSF protein were compared by a two-tailed Kruskal-Wallis test with Dunn’s correction for multiple comparisons. The patients with CNTN1 and CNTN1/Caspr1 antibodies were included in previous studies.11 13
*p<0.05, **p<0.01, *** p<0.001.
Caspr1, contactin-associated protein; CNTN1, contactin-1; CSF, cerebrospinal fluid; GBS, Guillain-Barré syndrome; mRS, modified Rankin Scale.