Study to Assess the Safety, Tolerability, Pharmacokinetics, and Effect on Disease Progression of BIIB078 Administered to Previously Treated Adults C9ORF72-Associated Amyotrophic Lateral Sclerosis (ALS)

NCT ID: NCT04288856

Last Updated: 2023-04-18

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

TERMINATED

Clinical Phase

PHASE1

Total Enrollment

75 participants

Study Classification

INTERVENTIONAL

Study Start Date

2020-04-28

Study Completion Date

2022-05-03

Brief Summary

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The primary objective is to evaluate the long-term safety and tolerability of BIIB078 in participants with chromosome 9 open reading frame 72-amyotrophic lateral sclerosis (C9ORF72-ALS).

The secondary objective is to evaluate the pharmacokinectic (PK) of BIIB078 in participants with C9ORF72-ALS.

Detailed Description

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This study is an extension study of NCT03626012.

Conditions

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Amyotrophic Lateral Sclerosis

Study Design

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Allocation Method

NON_RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

During the blinded loading period, the following individuals will be masked:

* Investigator
* Study staff
* Participants

After the loading period has been completed, subsequent doses will be open-label.

Study Groups

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Cohort A: BIIB078 First Dosage

BIIB078 will be administered as 3 doses during the loading period, approximately 2 weeks apart, and maintenance doses, approximately 4 weeks apart, via IT infusion.

Group Type EXPERIMENTAL

BIIB078

Intervention Type DRUG

Administered as specified in the treatment arm.

Cohort B: BIIB078 Second Dosage

BIIB078 will be administered as 3 doses during the loading period, approximately 2 weeks apart, and maintenance doses, approximately 4 weeks apart, via IT infusion.

Group Type EXPERIMENTAL

BIIB078

Intervention Type DRUG

Administered as specified in the treatment arm.

Cohort C: BIIB078 Third Dosage

BIIB078 will be administered as 3 doses during the loading period, approximately 2 weeks apart, and maintenance doses, approximately 4 weeks apart, via IT infusion.

Group Type EXPERIMENTAL

BIIB078

Intervention Type DRUG

Administered as specified in the treatment arm.

Possible Cohort D: BIIB078 Fourth Dosage

BIIB078 will be administered as 3 doses during the loading period, approximately 2 weeks apart, and maintenance doses, approximately 4 weeks apart, via IT infusion.

Group Type EXPERIMENTAL

BIIB078

Intervention Type DRUG

Administered as specified in the treatment arm.

Interventions

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BIIB078

Administered as specified in the treatment arm.

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

* Participants must have completed study NCT03626012 through the first follow-up clinic visit that follows the final dosing visit without missing more than 1 dose of study treatment.
* Participants taking concomitant riluzole at study entry must be on a stable dose for ≥30 days prior to the first dose of study treatment (Day 1). Participants taking concomitant riluzole must be willing to continue with the same dose regimen throughout the study, unless the Investigator determines that riluzole should be discontinued for medical reasons, in which case it may not be restarted during the study.
* Participants taking concomitant edaravone at study entry must be on a stable dose for ≥60 days prior to the first dose of study treatment (Day 1). Participants taking concomitant edaravone must be willing to continue with the same dose regimen throughout the study, unless the Investigator determines that edaravone should be discontinued for medical reasons, in which case it may not be restarted during the study. Edaravone may not be administered on dosing days of this study.

Exclusion Criteria

* History of drug abuse or alcoholism ≤6 months before study enrollment that would limit participation in the study, as determined by the Investigator.
* Presence of an implanted shunt for the drainage of CSF or an implanted central nervous system (CNS) catheter.
* History of or positive test result at Screening for human immunodeficiency virus. The requirement for testing at Screening may be omitted if it is not permitted by local regulations.
* Treatment with another investigational drug (including investigational drugs for ALS through compassionate use programs) or biological agent within 1 month of Screening or 5 half-lives of study agent, whichever is longer.
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Biogen

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Medical Director

Role: STUDY_DIRECTOR

Biogen

Locations

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Research Site

La Jolla, California, United States

Site Status

Research Site

Los Angeles, California, United States

Site Status

Research Site

Palo Alto, California, United States

Site Status

Research Site

Jacksonville, Florida, United States

Site Status

Research Site

Miami, Florida, United States

Site Status

Research Site

Atlanta, Georgia, United States

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Research Site

Baltimore, Maryland, United States

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Research Site

Boston, Massachusetts, United States

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Research Site

St Louis, Missouri, United States

Site Status

Research Site

Lincoln, Nebraska, United States

Site Status

Research Site

New York, New York, United States

Site Status

Research Site

Knoxville, Tennessee, United States

Site Status

Research Site

Calgary, Alberta, Canada

Site Status

Research Site

Edmonton, Alberta, Canada

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Toronto, Ontario, Canada

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Montreal, Quebec, Canada

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Research Site

Utrecht, , Netherlands

Site Status

Research Site

Sankt Gallen, , Switzerland

Site Status

Research Site

London, Greater London, United Kingdom

Site Status

Research Site

London, Greater London, United Kingdom

Site Status

Research Site

Sheffield, South Yorkshire, United Kingdom

Site Status

Countries

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United States Canada Netherlands Switzerland United Kingdom

Other Identifiers

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2019-004798-14

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

245AS102

Identifier Type: -

Identifier Source: org_study_id

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