Table 1

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 NF15595% CIOR vs seronegative95% CI
Initial clinical diagnosis of GBS5/8 (63%)3/15 (20%)3/11 (27%)4/7 (57%)38/185 (21%)6.71.1 to 34.56.51.6 to 24.9
Acute/subacute progression8/8 (100%)7/15 (47%)4/11 (36%)5/7 (71%)56/184 (30%)2.0 to ∞4.8 to ∞
Ataxia3/8 (38%)7/15 (47%)7/11 (64%)5/7 (71%)62/158 (39%)0.70.1 to 3.40.90.2 to 3.7
Tremor0/8 (0%)5/15 (33%)3/11 (27%)3/7 (43%)39/154 (25%)00 to 1.300 to 1.4
Neuropathic pain4/8 (50%)1/15 (7%)7/11 (64%)5/7 (71%)49/134 (37%)141.3 to 1801.70.5 to 6.2
Cranial nerve palsy8/8 (100%)5/15 (33%)5/11 (45%)1/7 (14%)41/156 (26%)3.3 to ∞5.7 to ∞
Autonomic dysfunction6/8 (75%)0/15 (0%)2/11 (18%)0/29/71 (13%)6.1 to ∞20.73.9 to 105.4
Respiratory involvement7/8 (88%)0/15 (0%)3/11 (27%)0/725/185 (14%)9.9 to ∞44.87.3 to 506.3
Nephrotic syndrome3/8 (38%)0/15 (0%)9/11 (82%)0/75/147 (3%)1.9 to ∞173.5 to 73.7
MRI plexus/root abnormalities2/4 (50%)2/7 (29%)2/7 (29%)5/6 (83%)15/53 (28%)6.71.1 to 34.56.51.6 to 24.9
Nadir mRS >48/8 (100%)3/15 (20%)4/11 (36%)2/7 (29%)38/185 (21%)
Significant vs NF155P valueSignificant vs seronegativeP 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.