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First in Human (FIH) Study of ALN-SOD in Adult Participants with Amyotrophic Lateral Sclerosis Associated with Mutation in the SOD1 Gene (SOD1-ALS)

Phase 1/2
Recruiting
Conditions
Amyotrophic lateral sclerosis (ALS)
Registration Number
2023-510344-20-00
Lead Sponsor
Regeneron Pharmaceuticals Inc.
Brief Summary

To evaluate the safety and tolerability of single and repeated IT ALN-SOD administration in participants with ALS associated with mutation in the SOD1 gene (SOD1-ALS)

Detailed Description

Not available

Recruitment & Eligibility

Status
RECRUITING
Sex
Not specified
Target Recruitment
13
Inclusion Criteria

Weakness attributable to ALS and a SOD1 mutation that has been previously described as associated with ALS or is considered likely to cause ALS, as defined in the protocol.

Slow vital capacity (SVC) ≥50% predicted value based on age, gender and height, measured in upright position.

Body Mass Index (BMI) ≤35 kg/m2 at time of screening.

If participants are taking riluzole, edaravone, or AMX0035, they must be on a stable dose for at least 4 weeks prior to initial dosing visit and are expected to remain at that dose until the end of the study.

Platelet count >50,000/microliter.

Has normal blood pressure readings, as defined in the protocol.

Other protocol defined inclusion criteria apply

Exclusion Criteria

Concurrent participation in another interventional clinical trial.

Has had a tracheostomy.

Has dementia, as assessed by the investigator.

Has uncontrolled psychiatric disease, including psychosis, active or recent suicidal ideation, untreated major depression, in the past 30 days.

Has a medical history of brain or spinal disease/injury that would interfere with the lumbar puncture (LP) process, CSF circulation or safety assessment, as defined in the protocol.

Presence of an implanted shunt for the drainage of CSF or an implanted central nervous system (CNS) catheter.

Presents any concern to the study investigator that might confound the results of the study or poses an additional risk to the participant by their participation in the study.

Was hospitalized (ie, >24 hours) for any reason other than ALS within 30 days of the screening visit.

Other protocol defined exclusion criteria apply

Study & Design

Study Type
INTERVENTIONAL
Study Design
SEQUENTIAL
Primary Outcome Measures
NameTimeMethod
Incidence of treatment-emergent adverse event (TEAEs) in participants treated with ALN-SOD

Incidence of treatment-emergent adverse event (TEAEs) in participants treated with ALN-SOD

Severity of TEAEs in participants treated with ALN-SOD

Severity of TEAEs in participants treated with ALN-SOD

Secondary Outcome Measures
NameTimeMethod
Incidence of anti-drug antibodies (ADAs) to ALN-SOD in serum

Incidence of anti-drug antibodies (ADAs) to ALN-SOD in serum

Titer of ADAs to ALN-SOD in serum

Titer of ADAs to ALN-SOD in serum

Concentration of ALN-SOD in plasma

Concentration of ALN-SOD in plasma

Concentration of ALN-SOD in CSF

Concentration of ALN-SOD in CSF

Concentration of SOD1 protein in cerebrospinal fluid (CSF)

Concentration of SOD1 protein in cerebrospinal fluid (CSF)

Change in concentration of SOD1 protein in CSF

Change in concentration of SOD1 protein in CSF

Concentration of neurofilament light chain (NfL) in plasma

Concentration of neurofilament light chain (NfL) in plasma

Change in concentration of NfL in plasma

Change in concentration of NfL in plasma

Concentration of NfL in CSF

Concentration of NfL in CSF

Change in concentration of NfL in CSF

Change in concentration of NfL in CSF

Trial Locations

Locations (14)

Macquarie University

🇦🇺

Sydney, New South Wales, Australia

Concord Repatriation General Hospital

🇦🇺

Concord, New South Wales, Australia

Sunshine Coast University Hospital

🇦🇺

Birtinya, Queensland, Australia

University of Alberta Hospital, Edmonton, Division of Neurology

🇨🇦

Edmonton, Alberta, Canada

University Hospital - London Health Sciences Centre

🇨🇦

London, Ontario, Canada

Sunnybrook Research Institute

🇨🇦

Toronto, Ontario, Canada

Montreal Neurological Institute and Hospital

🇨🇦

Montreal, Quebec, Canada

Hokkaido University Hospital

🇯🇵

Sapporo, Hokkaido, Japan

Tokushima University Hospital

🇯🇵

Tokushima, Tokushima, Japan

Toho University Omori Medical Center

🇯🇵

Ōta-ku, Tokyo, Japan

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Macquarie University
🇦🇺Sydney, New South Wales, Australia

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