Comparison of immunotherapy response and outcome in paediatric patients with antibody positive and antibody negative encephalopathy
| Antibody positive (n=21) | Antibody negative (n=27) | |
|---|---|---|
| Immunotherapy received | 17 (80%) | 17 (63%) |
| Corticosteroids only | 6 | 11 |
| IVIG only | 0 | 2 |
| Corticosteroids+IVIG | 11 | 4 |
| Additional PLEX | 4 | 0 |
| Disease modifying drugs | 5 | 0 |
| Immunotherapy response | 16 (94%) | 16 (94%) |
| Probable response | 10 | 13 |
| Definite response | 6 | 3 |
| Modified Rankin scale score (for children) at nadir | 4.5+0.60 | 4.5+0.58 |
| Modified Rankin scale score (for children) at follow-up | 1.8+0.75 | 1.6+0.84 |
| Ongoing problems (further details in figure 3) | 15 (71%) | 13 (48%) |
| 10/14 (71%) untreated | 3/4 (75%) | 13 (48%) |
| 18/34 (52%) treated | 12/17 (70%) | 6/17 (35%) |
No significant difference was seen in immunotherapy response and outcome. Antibody positive patients were more likely to receive PLEX and second line immunotherapy.
IVIG, intravenous immunoglobulins; PLEX, plasma exchange.