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

1. A method of treating or delaying the progression of a central nervous system (CNS) disorder alleviated by inhibiting complement immune system activation in a patient in need of such treatment, the method comprising administering to said patient at the onset of an active CNS attack one or more doses of at least 2000 U of C1-esterase inhibitor (C1-INH) per dose, wherein said disorder is neuromyelitis optica (NMO) or neuromyelitis optica spectrum disorder (NMOSD).

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 the specific brain areas of the hypothalamus, periventricular nucleus, or brainstem, and NMO-IgG negative NMO:AQP4 antibody-seronegative NMO.

4. The method according to claim 1 , wherein said disorder is NMO.

5. A method according to claim 1 , wherein said administration is during the early acute phase.

6. A method according to claim 1 , wherein said administration is of short term duration.

7. The method according to claim 1 , further comprising administering an adjunct treatment 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 the specific brain areas of the hypothalamus, periventricular nucleus, or brainstem, and NMO-IgG negative NMO:AQP4 antibody-seronegative NMO.

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

9. The method according to claim 8 , wherein the intravenous immune therapy comprises a glucocorticosteroid.