Table 1. Demographic, clinical, therapeutic and outcome data in the total cohort and stratified by age.
| Total cohort | Paediatric onset | Adult onset | Standardised mean difference | ||
| n | 109 | 41 | 68 | – | |
| Onset age, years median (IQR) | 26 (8–38) | 7 (5–9) | 34 (29–46) | 2.99 | |
| Sex assigned at birth, n females (%) | 62 (57) | 25 (61) | 37 (54) | 0.13 | |
| Disease course, n of relapsing patients (%) | 76 (70) | 28 (68) | 48 (71) | 0.05 | |
| Duration of follow-up, years median (IQR) | 6.2 (2.6–9.6) | 5.9 (2.7–10.1) | 6.3 (2.6–9.2) | 0.02 | |
| Onset episode phenotypen of patients (%) | Isolated optic neuritis (% of total phenotypes) | 58 (53) | 15 (37) | 43 (63) | 0.55 |
| Bilateral (n) | 35 | 10 | 25 | 0.26 | |
| Unilateral (n) | 23 | 5 | 18 | – | |
| Isolated transverse myelitis (TM) (% of total cohort) | 10 (9) | 1 (2) | 9 (13) | 0.38 | |
| Longitudinally extensive (n) | 4 | 1 | 3 | 0.1 | |
| Short segment only (n) | 6 | 0 | 6 | – | |
| Acute disseminated encephalomyelitis | 19 (17) | 19 (46) | 0 | 1.5 | |
| Cerebral cortical encephalitis | 2 (2) | 1 (2) | 1 (1) | 0.07 | |
| Mixed phenotypes* | 19 (17) | 5 (12) | 14 (21) | 0.22 | |
| Onset episode treatmentn of patients (%) | Nil | 18 (17) | 5 (12) | 13 (19) | 0.19 |
| IV corticosteroids only | 26 (24) | 4 (10) | 22 (32) | 0.54 | |
| Oral corticosteroids only | 12 (11) | 6 (15) | 6 (9) | 0.18 | |
| IV and oral corticosteroids | 53 (49) | 26 (63) | 27 (40) | 0.48 | |
| Initial oral corticosteroid dose (mg/kg) | 0.96 (0.65–1.31) | 1.28 (1.04–1.92) | 0.65 (0.65–0.65) | 1.78 | |
| Corticosteroids and IVIg/PLEX | 9 (8) | 5 (12) | 4 (6) | 0.23 | |
| Maintenance treatment instituted at disease onset | IVIg/PLEX | 0 | 0 | 0 | – |
| Other immunotherapies† | 5 (5) | 0 | 5 (7) | 0.35 | |
Data are summarised as n (%), or median (IQR). disseminated encephalomyelitis.
Other presentation phenotypes: 1 UON+STM (adult); 1 UON+LETM (paediatric); 1 BON+LETM (adult);1 UON+STM+area postrema syndrome (adult); 1 BON+LETM+diencephalic+Iintracranial hypertension; 4 Ccerebral syndrome (adults); 1 BON+LGI1 antibody encephalitis (adult); 1 ADEM+BON (paediatric); 1 ON+cerebellum (adult); 1 TM+cerebellum (adult); 1 LETM+Ccerebral (adult); 2 LETM+brainstem (adults); 1 CCE+brainstem (adult); 1 brainstem+cerebellum (paediatric); 1 Aaseptic meningitis (adult).
Other maintenance immunotherapies: 3 rituximab; 11 azathioprine; 11 mycophenolate mofmofetil.
ADEMacute disseminated encephalomyelitisBON, bilateral optic neuritis; CCE, cerebral cortical encephalitis; IVintravenousIVIg, Intravenous immunoglobulin; LETM, longitudinally extensive transverse myelitis; LGI1, Leucine rich glioma-inactivated 1; PLEX, Therapeutic plasma exchange; STM, short transverse myelitis; UON, unilateral optic neuritis