Table 4.
Management of Neuromyelitis Optica
| Medication | Use | Typical dose | Evidence |
|---|---|---|---|
| High dose IV methylprednisolone | Acute | 1 gm IV daily for 5 days with or without a taper | Observational studies |
| Plasma exchange | Acute as a rescue therapy | 5 exchanges (each exchange 250 ml) over 5-10 days | Randomized trials in TM patients |
| Rituximab | Maintenance | 1 gm (or 375 mg/m2) IV every 1-2 weeks for 2-4 weeks then redoes based on CD19 count (typically every 6-8 month) for ≤ 2 years | Several open label and retrospective clinical trials |
| Azathioprine | Maintenance | 2 mg/kg PO divided BID (typically 100 mg BID) for ≤2 years | Observational studies |
| Mycophenolate | Maintenance | 1-3 gm PO daily divided BID or TID for ≤2 years | Retrospective trial |
| Methotrexate | Maintenance | 5-15 mg PO weekly for ≤2 years | Open label trial |
| Mitoxantrone | Maintenance | 12 mg/m2 every 3 months (maximum dose 140 mg/m2) | Open label trial |
| Cyclophosphamide | Maintenance | 0.5-1.5 mg/m2 (typically 1 gm) IV every month until absolute lymphocyte count<1000/mm3 (typically 6 cycles) or immunoablative dose of 200 mg/kg divided over 4 days | Open label trial |
| IVIG | Maintenance | 2 gm/kg induction followed by 0.4-0.5 gm/kg every month | Case series |