Table 7.
Secondary analysis of opsoclonus-myoclonus syndrome (OMS): international group (N = 31).
| Feature | Data |
||
|---|---|---|---|
| Median (interquartile range) | N | % | |
| Patient age, years | 3.3 (2.2–4.7) | ||
| Age of OMS onset, years | 1.5 (1–1.8) | ||
| OMS duration, years | 1.4 (0.62–3.4) | ||
| Total score (OMS severity) (N = 29) | 16 (11–25) | ||
| Untreated | (13, 20) | 2 | 7 |
| Arriving on treatment | 15 (10–24) | 19 | 65 |
| Treated previously only | 16 (10–26) | 8 | 28 |
| OMS severity category | |||
| Mild (TS 0–12) | 8 | 28 | |
| Moderate (TS 12–24) | 12 | 41 | |
| Severe (TS 25–36) | 9 | 31 | |
| OMS duration category | |||
| Acute (≤3 months) | 2 | 6 | |
| Subacute (>3 to <12 months) | 8 | 26 | |
| Chronic (≥12 months) | 21 | 68 | |
| Tumor | |||
| Yes | 21 | 68 | |
| No | 10 | 32 | |
| Tumor type | |||
| NB | 10 | 59 | |
| GNB | 5 | 29 | |
| GN | 2 | 12 | |
| Not available | 4 | – | |
| CSF OCB (N = 25) | |||
| Positive (≥2) | 14 | 56 | |
| Negative (<2) | 11 | 44 | |
| CSF % B cells (N = 27) | |||
| Positive (≥2%) | 21 | 78 | |
| Negative (<2%) | 6 | 22 | |
| Treatment category (N = 29) | |||
| 1 agent | 5 | 17 | |
| 2 agents | 12 | 41 | |
| 3 agents | 7 | 24 | |
| ≥3 agents | 5 | 17 | |
| Treatment agents | |||
| Corticosteroids | 22 | ||
| Prednisone | 3 | ||
| Prednisolone | 2 | ||
| Methylprednisolone | 1 | ||
| Dexamethasone | 5 | ||
| Deflazacort | 1 | ||
| Corticotropin (1–39) | 11 | ||
| Corticotropin (1–24) | 4 | ||
| Intravenous immunoglobulin | 22 | ||
| CPM | 5 | ||
| RTX | 3 | ||
| Chemo | 9 | ||
| Azathioprine | 2 | ||
| Study period | |||
| First | 1 | 3 | |
| Second | 5 | 16 | |
| Third | 25 | 81 | |
There were only two untreated patients, no median value.
Of patients treated previously only: 5 had received a single agent; 22, more than one agent; 4, untreated. Of patients arriving on treatment: 10 on single agent, 10 on more than one agent.
Deflazacort: 6 mg is equivalent to 5 mg of prednisolone.