NCT05659901

Brief Summary

The purpose of the study is to prospectively assess longitudinal changes in proteolipid protein 1 (PLP1) protein, disease-related biomarkers in cerebral spinal fluid (CSF) and blood, neuroimaging parameters relevant to Pelizaeus-Merzbacher disease (PMD) and longitudinal changes in performance on clinical, participant, and caregiver-reported outcome assessments to inform the development of therapies for PMD.

Trial Health

83
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
32

participants targeted

Target at P25-P50 for all trials

Timeline
34mo left

Started Oct 2022

Longer than P75 for all trials

Geographic Reach
7 countries

9 active sites

Status
recruiting

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

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Study Timeline

Key milestones and dates

Study Progress56%
Oct 2022Mar 2029

Study Start

First participant enrolled

October 3, 2022

Completed
2 months until next milestone

First Submitted

Initial submission to the registry

November 22, 2022

Completed
29 days until next milestone

First Posted

Study publicly available on registry

December 21, 2022

Completed
6.2 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

March 1, 2029

Expected
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

March 1, 2029

Last Updated

April 17, 2026

Status Verified

April 1, 2026

Enrollment Period

6.4 years

First QC Date

November 22, 2022

Last Update Submit

April 16, 2026

Conditions

Keywords

PMD

Outcome Measures

Primary Outcomes (3)

  • Assess Longitudinal Changes in Fluid Biomarkers

    Changes in Proteolipid Protein 1 (PLP1) in CSF and disease related biomarkers

    Up to 26 months

  • Assess Longitudinal Changes in Neuroimaging Parameters

    Changes in regional brain volumes (MRI) and in brain metabolites (MRS)

    Up to 26 months

  • Assess longitudinal changes in performance on clinical, and patient and caregiver-reported outcome assessments

    Includes collection of gross and fine motor outcomes, spasticity, dysphagia, cognition and behavior, and sleep.

    Up to 26 months

Secondary Outcomes (1)

  • Characterize health service utilization and economic and disease burden

    Up to 26 months

Study Arms (1)

Pelizaeus-Merzbacher Disease Participants

Participants will undergo CSF collection and neuroimaging procedures, up to Week 106 as a part of prospective study. Each participant's medical and family history data will be collected retrospectively from available medical notes and charts, from birth up to the end of the study period (up to 26 months).

Eligibility Criteria

Age6 Months - 17 Years
Sexmale(Gender-based eligibility)
Healthy VolunteersNo
Age GroupsChild (0-17)
Sampling MethodNon-Probability Sample
Study Population

Pelizaeus-Merzbacher disease participant population

You may qualify if:

  • Participant has a parent or caregiver capable of providing informed consent (signed and dated) and able to attend all scheduled study visits and provide feedback regarding the participant's symptoms and performance as described in the protocol and be able to comply with all study requirements
  • Participant has a diagnosis of Pelizaeus-Merzbacher Disease with genetic confirmation of PLP1 duplication
  • Male, 6 months-17 years old, inclusive, at the time of informed consent and phenotype consistent with classic PMD
  • No contraindications for lumbar punctures (LPs), blood draws, neuroimaging, sedation (if necessary) or other study procedures

You may not qualify if:

  • Clinically significant abnormalities in medical history or physical examination
  • \> 2 copies of the PLP1 gene

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (9)

Ionis Investigative Site

Atlanta, Georgia, 30342, United States

RECRUITING

Ionis Investigative Site

Philadelphia, Pennsylvania, 19104, United States

RECRUITING

Ionis Investigative Site

Clermont-Ferrand, France

ACTIVE NOT RECRUITING

Ionis Investigative Site

Le Kremlin-Bicêtre, 94270, France

ACTIVE NOT RECRUITING

Ionis Investigative Site

Göttingen, Germany

ACTIVE NOT RECRUITING

Ionis Investigative Site

Tel Aviv, 6423906, Israel

RECRUITING

Ionis Investigative Site

Milan, 20154, Italy

RECRUITING

Ionis Investigative Site

Amsterdam, 1081 HV, Netherlands

RECRUITING

Ionis Investigative Site

Leeds, LS1 3EX, United Kingdom

RECRUITING

MeSH Terms

Conditions

Pelizaeus-Merzbacher Disease

Condition Hierarchy (Ancestors)

Hereditary Central Nervous System Demyelinating DiseasesBrain Diseases, Metabolic, InbornBrain Diseases, MetabolicBrain DiseasesCentral Nervous System DiseasesNervous System DiseasesLeukoencephalopathiesDemyelinating DiseasesGenetic Diseases, X-LinkedGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesMetabolism, Inborn ErrorsMetabolic DiseasesNutritional and Metabolic Diseases

Central Study Contacts

Study Design

Study Type
observational
Observational Model
COHORT
Time Perspective
PROSPECTIVE
Sponsor Type
INDUSTRY
Responsible Party
SPONSOR

Study Record Dates

First Submitted

November 22, 2022

First Posted

December 21, 2022

Study Start

October 3, 2022

Primary Completion (Estimated)

March 1, 2029

Study Completion (Estimated)

March 1, 2029

Last Updated

April 17, 2026

Record last verified: 2026-04

Data Sharing

IPD Sharing
Will share

Ionis may share anonymized individual participant data, aggregated clinical data, and other types of data that support the results in this study. Data requests from qualified researchers will be considered once all three of the following criteria are met: (1) 12 months from marketing approval of the study drug in both the United States and European Union; (2) 18 months from conclusion of the study; and (3) 6 months from publication of study article. Access would be via a secure environment and is contingent upon approval of a research proposal and entry into an appropriate data use agreement. Requests to access data can be submitted via the website https://vivli.org/ourmember/ionis/.

More information

Locations