Compositions and methods useful for the treatment of neuromyelitis optica spectrum disorders

1. A method of treating or delaying the progression of neuromyelitis optica (NMO) or neuromyelitis optica spectrum disorder (NMOSD) in a patient in need of treatment, the method comprising administering to said patient during an active CNS attack one or more doses of at least 2000 U of C1-esterase inhibitor (C1-INH) per dose, and wherein the one or more doses decreases symptoms of NMO or NMOSD to pre-attack levels.

2. The method according to claim 1 , wherein the C1-INH is a human plasma-derived C1-INH or a recombinant C1-INH.

3. The method according to claim 1 , wherein the NMOSD is single or recurrent events of longitudinally extensive transverse myelitis, bilateral simultaneous or recurrent optic neuritis, Asian optic-spinal multiple sclerosis, optic neuritis associated with systemic autoimmune disease, optic neuritis or myelitis associated with lesions in specific brain areas comprising at least one of the hypothalamus, periventricular nucleus, and brainstem, or NMO-IgG negative NMO:AQP4 antibody-seronegative NMO.

4. The method of claim 1 , wherein the one or more doses of at least 2000 U of C1-INH is administered within 7 days from onset of the active CNS attack.

5. The method of claim 4 , wherein the one or more doses of at least 2000 U of C1-INH is administered within 72 hours from onset of the active CNS attack.

6. The method of claim 5 , wherein the one or more doses of at least 2000 U of C1-INH is administered within 24 hours from onset of the active CNS attack.

7. The method according to claim 1 , further comprising administering to said patient an adjunct treatment effective for treating or delaying the progression of NMO or NMOSD.

8. The method according to claim 7 , wherein the C1-INH and the adjunct treatment are administered to said patient concurrently or sequentially.

9. The method according to claim 7 , wherein said adjunct treatment is selected from intravenous immune therapy, plasmapheresis, administration of mycophenolate, administration of rituximab, administration of eculizumab, administration of intravenous immunoglobulin preparations, or a combination thereof.

10. The method according to claim 9 , wherein the C1-INH and the adjunct treatment are administered to said patient concurrently or sequentially.

11. The method according to claim 9 , wherein the intravenous immune therapy comprises intravenously administering a glucocorticoid.

12. The method according to claim 11 , wherein the C1-INH and the adjunct treatment are administered to said patient concurrently or sequentially.

13. The method according to claim 11 , wherein the glucocorticoid is methylprednisolone.

14. The method according to claim 12 , wherein the C1-INH is a human plasma-derived C1-INH.

15. The method according to claim 14 , wherein the glucocorticoid is methylprednisolone.

16. The method according to claim 12 , wherein the C1-INH is a recombinant C1-INH.

17. The method according to claim 16 , wherein the glucocorticoid is methylprednisolone.