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CompletedNCT03295071REALITYUpdated Mar 2, 2026

REALITY LHON Registry

An observational study in Leber Hereditary Optic Neuropathy, sponsored by GenSight Biologics. Completed at 11 sites in 5 countries. Per ClinicalTrials.gov, last updated 2026-03-02.

Sponsored by GenSight Biologics · Observational

Study type
Observational
Model
Cohort
Time perspective
Cross-sectional
Enrollment
44
Sex
All
01

Study summary

This study is a multi-country retrospective and cross-sectional observational study of affected LHON subjects, based on retrospective subjects' medical chart abstractions and cross-sectional administration of patient-reported outcomes (PROs).

Read the detailed description

The purpose of this study is to understand the evolution of visual functional and structural changes and other associated symptoms in patients with LHON. The relation between genetic, lifestyle and/or environmental factors and the LHON disease will be described, and a better understanding of the natural history of LHON disease and the healthcare associated to with the disease will be sought.

In addition, we would also like to understand the economic burden for patients and their families with LHON resulting from direct and indirect costs they may have because of their disease.

02

Conditions studied

  • Leber Hereditary Optic Neuropathy

Keywords

  • Heredity Optic Atrophy
  • Leber Hereditary Optic Atrophy
  • Leber Hereditary Optic Neuropathy
  • Eye Diseases
  • Hereditary Eye Diseases
  • Inherited retinal dystrophies or degeneration
  • Inborn Genetic Disease
  • Gene Therapy
  • Intravitreal Injections
  • Mitochondrial Disease
  • AAV2 Vectors
  • Nervous System Diseases
  • Neurodegenerative Disease
  • Heredodegenerative Disorders of the Nervous System
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Who can participate

Ages eligible
Child (0–17), Adult (18–64), Older adult (65+)
Sexes eligible
All
Accepts healthy volunteers
No
Sampling method
Probability sample

Study population

The study will recruit at least 50 affected LHON subjects (both adult and pediatric) from global clinical sites at the following countries, but not limited to: Spain, Italy, France, United Kingdom, and the United States. Efforts will be done to maintain the population of at least 50 affected LHON subjects.

Recruitment efforts of recruiting approximately 75% of eligible subjects with 11778/ND4 mutation and 30% of eligible subjects under the age of 18 at the time of index date, wherever possible, will be done at the study level.

Inclusion criteria

  • Subjects who have a confirmed and genotyped diagnosis of LHON;
  • Subjects with visual function outcomes data including at least 2 visual function assessments between 1 year and 3 years (+/- 4 weeks) after vision loss;
  • Subjects who are willing and able to provide written informed consent if required as per local regulations;
  • For LHON subjects under the age of 18 years, permission from a legal guardian to participate in the study;

Exclusion criteria

Exclusion Criteria:

  • Subjects who received any investigational drug, or participated in any LHON-related interventional clinical trial during the observational period;
  • Subjects without medical charts data available.
04

Study design

Observational model
Cohort
Time perspective
Cross-sectional
Enrollment
44 participants (actual)
Target follow-up
3 Years
Patient registry
Yes

Groups and cohorts

  • Single-group study

    This study is a multi-country retrospective and cross-sectional observational study of affected LHON subjects, based on retrospective subjects' medical chart abstractions and cross-sectional administration of patient-reported outcomes (PROs).

    Other: Patient-reported outcomes (PROs)

Interventions

  • OtherPatient-reported outcomes (PROs)

    Patient-reported outcomes (PROs)

05

What researchers measure

Primary outcomes

  1. Visual Function

    Visual function will include visual examination data from medical records.

    Time frame: All assessments available before enrollment

  2. The National Eye Institute Visual Function Questionnaire (VFQ)-25

    The National Eye Institute Visual Function Questionnaire (NEI-VFQ or VFQ)-25 is a valid and reliable 25-item version of the 51-item VFQ.

    Time frame: Enrollment

  3. The 36-Item Short Form Health Survey (SF-36)

    The SF-36 surveys health status and quality of life

    Time frame: Enrollment

  4. Child Health Questionnaire (CHQ)

    The CHQ uses the same structure and methodological approach as the SF-36 and is designed and normed for children from 5-to-18 years of age.

    Time frame: Enrollment

  5. EuroQol-5 Dimension (EQ-5D)-5L

    The EuroQol-5 Dimension-5 Level (EQ-5D-5L) is a generic HRQoL instrument that is widely used as a PRO measure.

    Time frame: Enrollment

Secondary outcomes

  1. Economic burden of disease

    Data will be gathered by questionnaire at cross-sectional survey. Direct costs and indirect costs resulting from LHON will be estimated by all enrolled subjects. Direct costs will include the medical and non-medical direct costs due to LHON

    Time frame: Enrollment

06

Study locations

11 sites
  • Doheny Eye Center UCLA Pasadena
    Pasadena, California 91105, United States
  • Emory University Hospital
    Atlanta, Georgia 30322, United States
  • Massachusetts Eye and Ear Infirmary
    Boston, Massachusetts 02114, United States
  • Wills Eye Institute
    Philadelphia, Pennsylvania 19107, United States
  • Alkek Eye Center
    Houston, Texas 77030, United States
  • CHU d'Angers
    Angers, 49100, France
  • CHNO Les Quinze Vingts
    Paris, 75012, France
  • Ospedale Bellaria
    Bologna, 40139, Italy
  • Ospedale San Raffaele
    Milan, 20132, Italy
  • Institut Catala de Retina
    Barcelona, 08022, Spain
  • Moorfields Eye Hospital
    London, Greater London EC1V 2PD, United Kingdom
07

References and documents

Publications

  • Carelli V, Newman NJ, Yu-Wai-Man P, Biousse V, Moster ML, Subramanian PS, Vignal-Clermont C, Wang AG, Donahue SP, Leroy BP, Sergott RC, Klopstock T, Sadun AA, Rebolleda Fernandez G, Chwalisz BK, Banik R, Girmens JF, La Morgia C, DeBusk AA, Jurkute N, Priglinger C, Karanjia R, Josse C, Salzmann J, Montestruc F, Roux M, Taiel M, Sahel JA; the LHON Study Group. Indirect Comparison of Lenadogene Nolparvovec Gene Therapy Versus Natural History in Patients with Leber Hereditary Optic Neuropathy Carrying the m.11778G>A MT-ND4 Mutation. Ophthalmol Ther. 2023 Feb;12(1):401-429. doi: 10.1007/s40123-022-00611-x. Epub 2022 Nov 30. PubMed 36449262 ↗
  • Newman NJ, Yu-Wai-Man P, Carelli V, Biousse V, Moster ML, Vignal-Clermont C, Sergott RC, Klopstock T, Sadun AA, Girmens JF, La Morgia C, DeBusk AA, Jurkute N, Priglinger C, Karanjia R, Josse C, Salzmann J, Montestruc F, Roux M, Taiel M, Sahel JA. Intravitreal Gene Therapy vs. Natural History in Patients With Leber Hereditary Optic Neuropathy Carrying the m.11778G>A ND4 Mutation: Systematic Review and Indirect Comparison. Front Neurol. 2021 May 24;12:662838. doi: 10.3389/fneur.2021.662838. eCollection 2021. PubMed 34108929 ↗
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Registry details

Key details

Study ID
NCT03295071
Lead sponsor
GenSight Biologics
Responsible party
Sponsor
First posted
Sep 27, 2017
Start date
Jan 3, 2018
Primary completion
Jul 1, 2020
Completion
Jul 1, 2020
Last update
Mar 2, 2026

Oversight

FDA-regulated drug
No
FDA-regulated device
No
View the source record on ClinicalTrials.gov ↗

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