Table 1

Comparison of demographic data, clinical features and outcome of 45 children with NMO or limited forms of the disease with MOG-IgG, AQP4-IgG or absent antibodies

MOG-IgG positive (n=25)AQP4-IgG positive (n=5)Seronegative (n=15)p Value
At baseline
BL MOG-IgG25 (100.0%)0 (0.0%)0 (0.0%)
Titre1280 (160–5120)*0 (0–20)0 (0–40)
BL AQP4-IgG0 (0.0%)5 (100.0%)0 (0.0%)
Titre0 (0)320 (20–5120)*0 (0)
Females13 (52.0%)4 (80.0%)6 (40.0%)0.298†
Age at onset (y)7 (2–15)*12 (6–14)*11 (1–17)*0.193*
Clinical FU (mo)33 (12–112)*27 (12–68)*40 (12–113)*0.604*
Serological FU (mo)20 (1–86)*16 (8–47)*25 (3–33)*0.990*
EDSS at BL5 (2–8.5)*4 (3–6.5)*4 (2–9)*0.803*
Initial diagnosis
 NMO6 (24.0%)0 (0.0%)0 (0.0%)0.004‡
 Unilateral ON7 (28.0%)0 (0.0%)5 (33.3%)
 BON5 (20.0%)1 (20.0%)2 (13.3%)
 LETM7 (28.0%)1 (20.0%)8 (53.3%)
 BS0 (0.0%)3 (60.0%)0 (0.0%)
Visual acuity at BL0.06 (0.02–0.7)*
n=15
0.2*
n=1
0.6 (0.01–0.8)*
n=5
0.440*
CSF OCB2 (8.0%)2 (40.0%)2/14 (14.3%)0.163†
CSF cells >4/µL13 (52.0%)5 (100.0%)6/14 (42.9%)0.082†
CSF cell count/µL17 (0–331)*28 (10–100)*2 (0–90)*0.108*
After 12 months
NMO7 (28.0%)2 (40.0%)1 (6.7%)0.010‡
Mono ON1 (4.0%)0 (0.0%)4 (26.7%)
BON4 (16.0%)0 (0.0%)2 (13.3%)
RON7 (28.0%)0 (0.0%)1 (6.7%)
LETM6 (24.0%)1 (20.0%)7 (46.7%)
BS0 (0.0%)2 (40.0%)0 (0.0%)
Monophasic 1 year14 (56.0%)1 (20.0%)13 (86.7%)0.018†
Relapses 1 year1 (1–4)*2 (1–4)*1 (1–3)*0.027*
At last follow-up
FU MOG-IgG17/23 (74%)0/5 (0.0%)0/6 (0.0%)
Titre160 (40–2560)*0 (0)0 (0–80)
FU AQP4-IgG0/23 (0.0%)4/5 (80.0%)0/6 (0.0%)
Titre0 (0)80 (0–5120)*0
EDSS at last FU0 (0–6)*1 (0–4)*1 (0–7)*0.146*
No ON ever6 (24.0%)2 (40.0%)7 (46.7%)0.402†
Unilateral ON ever9 (36.0%)2 (40.0%)6 (40.0%)
BON ever10 (40.0%)1 (20.0%)2 (13.3%)
Myelitis ever13 (52.0%)4 (80.0%)9 (60.0%)0.500†
LETM ever13 (52.0%)3 (60.0%)9 (60.0%)0.866†
Brainstem ever1 (4.0%)3 (60.0%)1 (6.7%)0.001†
Final diagnosis
 NMO7 (28.0%)3 (60.0%)2 (13.3%)0.169‡
 BON4 (16.0%)0 (0.0%)2 (13.3%)
 RON8 (32.0%)0 (0.0%)4 (26.7%)
 LETM6 (24.0%)1 (20.0%)7 (46.7%)
 BS0 (0.0%)1 (20.0%)0 (0.0%)
NMO
 Simultaneous6 (85.7%)0 (0.0%)0 (0.0%)0.015‡
 Sequential1 (14.3%)3 (100.0%)2 (100.0%)
Systems affected
 NMO7 (28.0%)3 (60.0%)2 (13.3%)0.042‡
 Isolated ON12 (48.0%)0 (0.0%)6 (40.0%)
 Isolated LETM6 (24.0%)1 (20.0%)7 (46.2%)
 Isolated brainstem0 (0%)1 (20.0%)0 (0.0%)
Monophasic13 (52.0%)0 (0.0%)8 (53.3%)0.095‡
Relapses1 (1–5)*2 (2–4)*1 (1–3)*0.107*
Recovery
 Complete17 (68.0%)3 (60.0%)6/14 (42.8%) 0.478‡
 Minor residuals7 (28.0%)2 (40.0%)6/14 (42.8%)
 Severe residuals1 (4.0%)0 (0.0%)2/14 (14.3%)
No therapy16 (64.0%)0 (0.0%)13 (86.7%)0.0002‡
 Immunosuppression3 (12.0%)5 (100.0%)2 (13.3%)
 Immunomodulation6 (24.0%)0 (0.0%)0 (0.0%)
Visual acuity at FU1.0 (0.8–1.0)*
n=18
1*
n=1
0.9 (0.3–1.0)*
n=8
0.207*
  • *Median (range), Kruskal-Wallis test.

  • †χ2 test.

  • ‡Corrected Fisher's exact contingency table analysis.

  • AQP4, aquaporin-4; BL, baseline; BON, bilateral optic neuritis; BS, brainstem syndrome; CSF, cerebrospinal fluid; EDSS, Expanded Disability Status Scale; FU, follow-up; LETM, longitudinally extensive transverse myelitis; MOG, myelin oligodendrocyte glycoprotein; NMO, neuromyelitis optica; OCB, oligoclonal band; ON, optic neuritis; RON, recurrent optic neuritis.