Dose-Escalation, Safety and Pharmacokinetic Study of IC14 in Motor Neurone Disease

Inclusion Criteria: A patient must fulfill all of the following criteria to be eligible for enrollment: 1. Signed informed consent prior to initiation of any study-specific procedures. 2. Familial or sporadic motor neurone disease (MND) defined as clinically possible, probable, or definite by Awaji-Shima Consensus Recommendations. 3. First symptoms of MND within 3 years of informed consent. 4. Age between 18 and 75 years at time of informed consent. 5. Seated Forced Vital Capacity (FVC) ≥ 65% of predicted value. 6. Not taking riluzole or on a stable dose of riluzole for at least 4 weeks prior to screening visit. 7. Adequate bone marrow reserve, renal and liver function: * absolute neutrophil count ≥ 1500/µL * lymphocyte count \< 48% * platelet count ≥ 150,000/µL * hemoglobin ≥ 11 g/dL * Estimated glomerular filtration rate (eGFR) \>= 40 mL/min/1.73 m2 * Alanine aminotransferase (ALT) and/or Aspartate aminotransferase (AST) ≤ 2x upper imit of normal (ULN), total bilirubin ≤ 1.5x ULN * serum albumin ≥ 2.8 g/dL 8. Females of childbearing potential should be using and committed to continue using one of the following acceptable birth control methods: * Sexual abstinence (inactivity) for 1 month prior to screening through study completion; or * Intrauterine device (IUD) in place for at least 3 months prior to study through study completion; or * Stable hormonal contraception for at least 3 months prior to study through study completion; or * Surgical sterilization (vasectomy) of male partner at least 6 months prior to study. 9. To be considered of non-childbearing potential, females should be surgically sterilized (bilateral tubal ligation, hysterectomy, or bilateral oophorectomy at least 2 months prior to study) or be post-menopausal and at least 3 years since last menses. 10. Males with female partners of childbearing potential must use contraception through study completion. 11. Medically safe to have lumbar puncture to collect cerebrospinal fluid. 12. Able to give informed consent and able to comply with all study visits and all study procedures. Exclusion Criteria: A patient fulfilling any of the following criteria at screening is to be excluded from enrollment in the study: 1. Dependence on mechanical ventilation, defined as being unable to lay supine without it, unable to sleep without it, or continuous daytime use; presence of tracheostomy at screening; or presence of diaphragm pacing system at screening. 2. Treatment with a drug or device within the last 30 days that has not received regulatory approval. 3. Treatment within 12 months with immunomodulator or immunosuppressant agent (including but not limited to cyclophosphamide, cyclosporine, interferon-α, interferon-β-1a, rituximab, alemtuzumab, azathioprine, etanercept, infliximab, adalimumab, certolizumab, golimumab, anakinra, rilonacept, secukinumab, tocilizumab, mycophenolate mofetil, methotrexate, haematopoietic stem cell transplantation). 4. Known active current or history of recurrent bacterial, viral, fungal, mycobacterial or other opportunistic infections or major episode of infection requiring hospitalization or treatment with IV antibiotics within 4 weeks. 5. History of severe allergic or anaphylactic reactions to human, humanized or murine monoclonal antibodies. 6. Presence of any of the following clinical conditions: * Bleeding diathesis or receipt of anticoagulants within 7 days (or any other clinical condition that would, in the opinion of the investigator, place the patient at increased risk during lumbar puncture). * History of one or more of the following: cardiac insufficiency (New York Heart Association \[NYHA\] III/IV), uncontrolled cardiac arrhythmias, unstable ischemic heart disease, or uncontrolled hypertension (systolic blood pressure \> 170 mmHg or diastolic blood pressure \> 110 mmHg). * History of venous thromboembolic disease within 12 months, myocardial infarction, or cerebrovascular accident. * Unstable pulmonary, renal, hepatic, endocrine or hematologic disease. * Autoimmune disease, mixed connective tissue disease, scleroderma, polymyositis, or significant systemic involvement secondary to rheumatoid arthritis. * Evidence of active malignant disease, malignancies diagnosed within the previous 5 years, or breast cancer diagnosed within the previous 5 years (except skin cancers other than melanoma). * Human immunodeficiency virus infection or other immunodeficiency illness. * Unstable psychiatric illness defined as psychosis or untreated major depression within 90 days. * Drug abuse or alcoholism within the past 12 months. * Significant neuromuscular disease other than MND. * Other ongoing disease that may cause neuropathy, such as toxin exposure, dietary deficiency, uncontrolled diabetes, hyperthyroidism, cancer, systemic lupus erythematosus or other connective diseases, infection with HIV, hepatitis B virus (HBV), or hepatitis C (HCV), Lyme disease, multiple myeloma, Waldenström's macroglobulinemia, amyloid, and hereditary neuropathy. 7. Pregnancy or breastfeeding. 8. Deprivation of freedom by administrative or court order.