MedPath

Efficacy & Safety Study of Bilateral IVT Injection of GS010 in LHON Subjects Due to the ND4 Mutation for up to 1 Year

Phase 3
Conditions
Leber Hereditary Optic Neuropathy
Interventions
Genetic: GS010
Drug: Placebo
Registration Number
NCT03293524
Lead Sponsor
GenSight Biologics
Brief Summary

The goal of this clinical trial is to assess the safety and efficacy of GS010, a gene therapy, in improving the retina functional \& structural outcomes in subjects with LHON due to the G11778A ND4 mitochondrial mutation when vision loss duration is present up to one year.

Detailed Description

GS-LHON-CLIN-05 is a Phase III, global, multi-center randomized, double-masked for the primary analysis, placebo-controlled, clinical study. As LHON is a neurodegenerative disease, the goal is to administer GS010 as soon as possible upon confirmation of the LHON diagnosis and the causative mutation.

Recruitment & Eligibility

Status
UNKNOWN
Sex
All
Target Recruitment
90
Inclusion Criteria
  • Vision loss duration of ≤ 365 days (i.e. ≤ 1 year) in each affected eye at Inclusion Visit (Visit 2).
  • Each eye of the subject must maintain at least Hand Motion (HM) visual acuity, as defined by the study's SOP for visual acuity testing.
  • Documented results of genotyping showing the presence of the G11778A mutation in the ND4 gene and the absence of the other primary LHON-associated mutations (ND1 or ND6) in the subject's mitochondrial DNA.

Main

Exclusion Criteria
  • Light Perception (LP) or No Light Perception (NLP) visual acuity in any eye, as defined by the study's standard operating procedure (SOP) for visual acuity testing.
  • Presence of active infectious conjunctivitis, keratitis, scleritis or endophthalmitis in either eye.
  • Presence of alcoholism, alcohol dependence, or alcohol or drug abuse (excluding nicotine).

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Treatment Arm 1GS010Subjects will be randomized to treatment arm 1 or treatment arm 2 in a 1:1 allocation. Subjects in treatment arm 1 will receive intravitreal GS010 in both eyes.
Treatment Arm 2PlaceboSubjects will be randomized to treatment arm 1 or treatment arm 2 in a 1:1 allocation. Subjects in treatment arm 2 will receive GS010 in one eye and placebo intravitreal injection in the other eye.
Treatment Arm 2GS010Subjects will be randomized to treatment arm 1 or treatment arm 2 in a 1:1 allocation. Subjects in treatment arm 2 will receive GS010 in one eye and placebo intravitreal injection in the other eye.
Primary Outcome Measures
NameTimeMethod
Best-Corrected Visual Acuity (BCVA) reported using Log of the Minimal Angle of Resolution (LogMAR) - 1 yearat 1.5 Year post baseline treatment

The primary efficacy endpoint will be the change from baseline (Visit 2) BCVA reported with LogMAR at 1.5 years post-treatment in second affected/not yet affected eyes of ND4 LHON subjects with vision loss up to one year. The change from baseline (Visit 2) in second affected/not yet affected eyes receiving GS010 and placebo will be the primary response of interest. LogMAR BCVA will be used to represent BCVA.

Secondary Outcome Measures
NameTimeMethod
Best-Corrected Visual Acuity (BCVA) reported with LogMAR - 2 yearsat 1.5-Year and 2-Years post baseline treatment

Change from baseline in LogMAR BCVA at each timepoint of the follow-up period and at 2 years post-treatment.

Spectral-Domain - Optical Coherence Tomography (SD-OCT) parameterat 1.5-Year and 2-Years post baseline treatment

Parameters measured with SD-OCT over time and at 1.5 and 2-years post baseline treatment. The change from baseline will be the response. Analysis will be performed mainly for second affected/not yet affected eyes treated with GS010 versus placebo and also for first affected eyes receiving GS010 as well

Humphrey Visual Field (HVF) parameterat 1.5-Year and 2-Years post baseline treatment

Parameters measured with HVF 30-2 over time and at 1.5 and 2-years post baseline treatment. The change from baseline will be the response. Analysis will be performed mainly for second affected/not yet affected eyes treated with GS010 versus placebo and also for first affected eyes receiving GS010 as well

Pelli Robson Low Vision Contrast Sensitivity parameterat 1.5-Year and 2-Years post baseline treatment

Parameters measured with Pelli Robson Low Vision Contrast Sensitivity over time and at 1.5 and 2-years post baseline treatment. The change from baseline will be the response. Analysis will be performed mainly for second affected/not yet affected eyes treated with GS010 versus placebo and also for first affected eyes receiving GS010 as well

Quality of Life: Visual Functioning Questionnaire-25at 1.5-Year and 2-Years post baseline treatment

Visual Functioning Questionnaire-25 at 1.5 and 2-years post-treatment

Responder Analysisat 1.5-Year and 2-Years post baseline treatment

Response status at each timepoint of the follow-up period and at 2 years post-treatment. Definitions of responder eyes include:

1. Eyes whose LogMAR BCVA improves (i.e. decreases) by ≥ 0.3 LogMAR (equivalent to a gain of ≥ 15 ETDRS letters) compared to baseline.

2. Eyes whose LogMAR BCVA does not increase (i.e. worsen) by ≥ 0.3 LogMAR (equivalent to eyes that lose ≤ 15 ETDRS letters) compared to baseline.

3. Eyes whose LogMAR visual acuity is \< 1.0 (i.e. better than LogMAR 1.0, equivalent to better than Snellen acuity of 20/200).

Quality of Life: 36-Item Short Form Health Survey, version 2 Questionnaireat 1.5-Year and 2-Years post baseline treatment

36-Item Short Form Health Survey, version 2 Questionnaire at 1 and 2-years post-treatment.

Trial Locations

Locations (13)

Massachusetts Eye and Ear Infirmary

🇺🇸

Boston, Massachusetts, United States

University of Colorado Health Eye Center

🇺🇸

Aurora, Colorado, United States

CHNO Les Quinze Vingts

🇫🇷

Paris, France

Doheny Eye Center UCLA Pasadena

🇺🇸

Pasadena, California, United States

Emory Healthcare - The Emory Clinic

🇺🇸

Atlanta, Georgia, United States

Icahn School of Medicine at Mount Sinai

🇺🇸

New York, New York, United States

Wills Eye Institute - Ocular Oncology Service

🇺🇸

Philadelphia, Pennsylvania, United States

Vanderbilt Eye Institute

🇺🇸

Nashville, Tennessee, United States

Moorfields Eye Hospital

🇬🇧

London, Greater London, United Kingdom

Hospital Universitario Ramon y Cajal

🇪🇸

Madrid, Spain

Taipei Veterans General Hospital

🇨🇳

Taipei, Taiwan

IRCCS Istituto delle Scienze Neurologiche di Bologna UOC Clinica Neurologica

🇮🇹

Bologna, Italy

Universitair Ziekenhuis Gent

🇧🇪

Gent, Belgium

© Copyright 2025. All Rights Reserved by MedPath