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

1. A method of treating or delaying the progression of a CNS disorder alleviated by inhibiting complement immune system activation in a patient in need of such treatment, the method comprising administering during an active CNS attack, a therapeutically effective amount of C1-esterase inhibitor (C1-INH), wherein said disorder is neuromyelitis optica (NMO) or neuromyelitis optica spectrum disorder (NMOSD), and wherein administering the therapeutically effective amount of C1-INH decreases symptoms of NMO or NMOSD to pre-attack levels.

2. The method according to claim 1 , wherein the C1-esterase inhibitor (C1-INH) comprises a human plasma-derived C1-INH (hC1-INH) or a recombinant C1-INH (rC1-INH).

3. The method according to claim 1 , wherein the disorder is selected from the group consisting of neuromyelitis optica (NMO) or Devic's disease, 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 such as the hypothalamus, periventricular nucleus, and brainstem, and NMO-IgG negative NMO: AQP4 antibody seronegative NMO.

4. The method of claim 3 , wherein said disorder is NMO and said C1 esterase inhibitor is CINRYZE®.

5. The method according to claim 1 , comprising administering an additional biologically active agent effective for treating or delaying the progression of a disorder selected from the group consisting of neuromyelitis optica (NMO) or Devic's disease, 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 such as the hypothalamus, periventricular nucleus, and brainstem, and NMO-IgG negative NMO: AQP4 antibody seronegative NMO.

6. The method according to claim 5 , wherein said treatment is plasmapheresis and/or administration of intravenous immunoglobulin preparations.

7. The method according to claim 6 , wherein the C1-INH and the biologically active agent are administered concurrently.

8. The method according to claim 6 , wherein the C1-INH and the biologically active agent are administered sequentially.

9. The method according to claim 5 , comprising administering mycophenolate, rituximab and/or eculizumab.

10. The method according to claim 9 , wherein the C1-INH and the biologically active agent are administered concurrently.

11. The method according to claim 9 , wherein the C1-INH and the biologically active agent are administered sequentially.

12. A method as claimed in claim 10 , wherein said administration is during the early acute phase.

13. A method as claimed in claim 10 , wherein said administration is of short term duration.

14. The method according to claim 5 , wherein the C1-INH and the biologically active agent are administered concurrently.

15. The method according to claim 5 , wherein the C1-INH and the biologically active agent are administered sequentially.

16. A method as claimed in claim 1 , wherein said administration is during the early acute phase.

17. A method as claimed in claim 1 , wherein said administration is of short term duration.

18. A method of preventing or delaying the progression of a CNS disorder alleviated by inhibiting complement immune activation in a patient in need of such treatment, the method comprising administration at the onset of an active CNS attack, a therapeutically effective amount of C1-esterase inhibitor (C1-INH), wherein said disorder is neuromyelitis optica (NMO) or neuromyelitis optica spectrum disorder (NMOSD), and wherein administering the therapeutically effective amount of C1-INH decreases symptoms of NMO or NMOSD to pre-attack levels.