Comparison of demographic, clinical features, outcome and MRI of 33 paediatric patients with ADEM with or without MOG-IgG antibodies
| MOG-IgG at baseline | |||
|---|---|---|---|
| negative (n=14) | positive (n=19) | p Value | |
| MOG-IgG titre (1:) at baseline | 0 (0) | 5120 (160–20 480) | |
| MOG-IgG at follow-up | 0/6 (0.0%) | 8/18 (44.4%) | |
| MOG-IgG titre (1:) at follow-up | 0 (0) | 80 (0–5120) | |
| Decrease of MOG-IgG titre ≥2 steps | 16/18 (88.9%) | ||
| CSF OCBs | 1 (7.1%) | 1/18 (5.6%) | 1.000 |
| CSF cell count/µL | 10 (1–95) | 53 (2–339) | 0.038 |
| Females | 6 (40.0%) | 9 (50.0%) | 1.000 |
| Age at onset (years) | 7 (1–13) | 4 (1–17) | 0.084 |
| Symptoms | |||
| Altered consciousness | 13 (92.9%) | 17 (89.5%) | 1.000 |
| Emotional/behaviour | 6 (42.9%) | 1 (5.3%) | 0.026 |
| Seizures | 2 (14.3%) | 2 (10.5%) | 1.000 |
| Optic neuritis | 2 (14.3%) | 3 (15.8%) | 1.000 |
| CN (III–XII) | 6 (42.9%) | 7 (36.8%) | 1.000 |
| Pyramidal signs | 3 (21.4%) | 3 (15.8%) | 1.000 |
| Ataxia | 5 (35.7%) | 10 (52.6%) | 0.482 |
| Sensory symptoms | 2 (14.3%) | 1 (5.3%) | 0.561 |
| Spinal symptoms | 6 (42.9%) | 10 (52.6%) | 0.728 |
| TM (symptom+MRI lesion) | 4 (28.6%) | 10 (52.6%) | 0.286 |
| Follow-up (months) | 25 (6–61) | 27 (5–92) | 1.000 |
| Multiphasic disease course | 0 (0.0%) | 4 (21.1%) | 0.119 |
| Clinical recovery | |||
| Complete | 9 (64.3%) | 15 (78.9%) | 0.038 |
| Minor residuals | 0 (0.0%) | 3 (15.8%) | |
| Severe residuals | 5 (35.7%) | 1 (5.3%) | |
| MRI areas affected | |||
| 1. Supratentoriell WM | 13 (92.9%) | 16 (84.2%) | 1.000 |
| 2. Thalamus/basal ganglia | 9 (64.3%) | 17 (89.5%) | 0.238 |
| 3. Brainstem | 8 (57.1%) | 14 (73.7%) | 0.459 |
| 4. Cerebellar | 5 (35.7%) | 12 (63.2%) | 0.166 |
| 5. Spinal | 4/12 (33.3%) | 13/14 (92.9%) | 0.003 |
| Widespread score (items 1–5) | 3 (1–5) | 4 (2–5) | 0.035 |
| Brainstem/spinal lesions combined | 8 (57.1%) | 18 (94.7%) | 0.026 |
| LETM | 4/12 (33.3%) | 13/14 (92.9%) | 0.003 |
| Bilateral lesions | 14 (100.0%) | 19 (100.0%) | 1.000 |
| Large lesions >2 cm axial | 13 (92.9%) | 19 (100.0%) | 0.424 |
| Atypical MRI features | |||
| Only one MRI area affected | 3 (21.4%) | 0 (0%) | 0.067 |
| Diffuse WM involvement | 1 (7.1%) | 0 (0.0%) | 0.424 |
| Small lesions | 2 (14.3%) | 0 (0.0%) | 0.090 |
| T1-hypointense lesions | 1 (7.1%) | 0 (0.0%) | 0.424 |
| Well defined borders | 5 (35.7%) | 0 (0.0%) | 0.008 |
| Perpendicular | 1 (7.1%) | 0 (0.0%) | 0.424 |
| Periventricular | 1 (7.1%) | 1 (5.3%) | 1.000 |
| Cortical lesions | 1 (7.1%) | 1 (5.3%) | 1.000 |
| Number of atypical features | 1 (1–3) | 0 (0–1) | 0.032 |
| ADEM with ≤1 atypical MRI feature | 8 (57.1%) | 19 (100.0%) | 0.003 |
| MRI outcome available from | 14/14 | 17/19 | |
| 1. Complete resolution | 3 (21.4%) | 10 (58.8%) | 0.036 |
| 2. Minor residuals | 3 (21.4%) | 4 (23.5%) | |
| 3. Moderate residuals | 1 (7.1%) | 2 (11.8%) | |
| 4. Marked residuals | 7 (50.0%) | 1 (5.9%) | |
ADEM, acute disseminating encephalomyelitis; CN, cranial nerves; CSF, cerebrospinal fluid; LETM, longitudinal extensive transverse myelitis; MOG, myelin oligodendrocyte glycoprotein; OCBs, oligoclonal bands; TM, transverse myelitis; WM, white matter.