Table 3.
Clinical, demographic, and phenotypic expert panel classification of the childhood CNS acquired demyelinating syndromes.
| CIS (n = 83) | ||||||
|---|---|---|---|---|---|---|
| ADEM (n = 40) | ON (n = 31) | TM (n = 26) | Other CIS (n = 26) | NMO (n = 2) | ||
| Age at presentation: median (IQR) | 5.3 (3.6-7.0) | 11.8 (9.0-13.9) | 12.6 (9.3-14.0) | 14.0 (9.5-14.5) | 6.4 and 14.8 years | |
| Sex (n = 125) | Male: Female (% female) 61: 64 (51%) | 24:16 (40%) | 15:16 (52%) | 11:15 (58%) | 11:15 (58%) | 0:2 (100%) |
| 37:46 (55%) | ||||||
| Ethnicity (n = 124/125) | White (n = 101) | 36 (90%) | 21 (68%) | 22 (85%) | 21 (81%) | 1 (50%) |
| Asian (n = 11) | 3 | 4 | 2 | 1 | 1 | |
| Black (n = 7) | 1 | 3 | 1 | 2 | 0 | |
| Chinese, mixed, other (n = 5) | 0 | 3 | 1 | 1 | 0 | |
| Clinical: | MRI: | |||||
| Monofocal | ≥ 1 asymptomatic lesion | 7 (23%) | 9 (35%) | 4 (15%) | ||
| no asymptomatic | 24 | 17 | 3 (12%) | |||
| Multifocal | ≥ 1 asymptomatic | 0 | 0 | 16 (62%) | ||
| no asymptomatic | 0 | 0 | 2 (8%) | |||
| Radiologically isolated | n/a | n/a | 1 | |||
| Spinal lesion(s) present: n (% ≥ 3 segments) | 8/12 (100%) | 1/8 (0) | 26/26 (62%) | 4/9 (75%) | 2/2 (100%) | |
| Death | 1/40 (2.5%) | 0 | 0 | 0 | 0 | |
| Seizures | 8/40 (20%) | 0 | 0 | 0 | 0 | |
| Optic neuritis: Unilateral: bilateral (% bilateral) | 0:3 (100%) | 22:9 (29%) | 0:0 | 0:1 | 1:1 (50%) | |
| Cerebellar signs: n (%) | 18 (45%) | 0 | 0 | 11 (42%) | 0 | |
| Brain stem signs: n (%) | 11 (28%) | 0 | 1 | 13 (50%) | 0 | |
| Pyramidal signs: n (%) | 24 (60%) | 0 | 0 | 10 (38%) | 0 | |
| Intensive care unit admissions; n = 12 (10%) | 8 (20%) | 0/31 (0%) | 3/26 (12%) | 1/26 (4%) | 0/2 | |
| Plasma exchange; n = 4 (3%) | 2 (5%) | 0/31 | 2/26 (8%) | 0/26 | 0/2 | |
| Intravenous corticosteroids | 37/40 (93%) | 23/31 (74%) | 25/26 (96%) | 18/26 (69%) | 2/2 (100%) | |
| Oral steroids | 27/40 (68%) | 21/31 (67%) | 16/26 (62%) | 11/26 (42%) | 2/2 (100%) | |
| Intravenous immunoglobulin | 6/40 (15%) | 0 | 7/26 (27%) | 1/26 (4%) | 0 | |
ADEM, acute disseminated encephalomyelitis; ON, optic neuritis; TM, transverse myelitis; CIS, clinically isolated syndrome; NMO, neuromyelitis optica; MRI, magnetic resonance imaging; IQR, interquartile range.