Inclusion Criteria

Inclusion criteria

1. Criteria for neuromyelitis optica:

1. acute transverse myelitis and optic neuritis occurring within 30 days of each
other followed by a second attack of either optic neuritis and/or acute
transverse myelitis at least 3 months following the heralding attack OR

2. acute transverse myelitis followed by optic neuritis at least 3 months later OR

3. optic neuritis followed by acute transverse myelitis at least 3 months later

2. Criteria for high risk for neuromyelitis optica:

1. recurrent idiopathic recurrent acute transverse myelitis with at least 3 months
time between each attack OR

2. recurrent bilateral simultaneous optic neuritis with at least 3 months time
between each attack.

- Subjects should also have no clinical evidence of disease outside the optic
nerve or spinal cord.

- In addition patients should have one major supportive criteria OR two minor
supportive criteria:

1. Negative brain MRI at onset (Does not meet criteria for multiple
sclerosis (Paty, 1998)

2. Spinal cord MRI with signal abnormality extending over ≥3 vertebral
segments

3. CSF pleocytosis of > 50 WBC/mm3 OR > 5 PMNs/mm3

- Minor supportive criteria:

1. Bilateral optic neuritis

2. Severe optic neuritis with fixed visual acuity worse than 20/200 in at
least one eye

3. Severe, fixed, attack-related weakness (MRC ≤2) in one or more limbs

- Subjects must have exhibited evidence of treatment failure, defined as at
least one attack of either acute transverse myelitis or optic neuritis
within six months of screening despite treatment within steroids or other
immunomodulatory drug for the preceding attack.

- Able and willing to give written informed consent and comply with the
requirements of the study protocol.

- Adequate renal function as indicated by normal serum sodium, potassium,
chloride, bicarbonate, creatinine, and blood urea nitrogen studies.

- Adequate liver function, as indicated by normal bilirubin and alkaline
phosphatase, and transaminases (AST and ALT) within 2X upper limit of
normal.

- Negative serum pregnancy test (for women of child bearing age).

- Men and women of reproductive potential must agree to use an acceptable
method of birth control during treatment and for twelve months (1 year)
after completion of treatment.

Exclusion Criteria:

1. Treatment with broad-spectrum immunosuppressant medications such as cyclophosphamide,
mitoxantrone, methotrexate, azathioprine, and cladribine, within 60 days of
screening.

2. Treatment with any investigational agent within 4 weeks of screening

3. Receipt of a live vaccine within 4 weeks prior to randomization

4. Previous Treatment with Rituximab (MabThera® / Rituxan®)

5. Prior antibody therapy

6. History of exposure to clioquinol

7. History of severe allergic or anaphylactic reactions to humanized or murine
monoclonal antibodies

8. History of HIV (positive HIV, HIV conducted during screening if applicable)

9. History of Hepatitis B and/or Hepatitis C (Hep B/C at screening)

10. History of recurrent significant infection or history of recurrent bacterial
infections

11. Known active bacterial, viral fungal mycobacterial, or other infection or any major
episode of infection requiring hospitalization

12. Ongoing daily steroid use

13. History of drug, alcohol, or chemical abuse within 6 months prior to screening

14. Pregnancy (a negative serum pregnancy test should be performed for all women of
childbearing potential within 7 days of treatment) or lactation

15. Concomitant malignancies or previous malignancies within the last five years, with
the exception of adequately treated basal or squamous cell carcinoma of the skin or
carcinoma in situ of the cervix.

16. History of psychiatric disorder