NCT04476862

Brief Summary

This is a multicenter, observational study for patients with a confirmed diagnosis of neuronal ceroid lipofuscinosis type 2 (CLN2 disease), also known as TPP1 deficiency, who intend to be or are currently being treated with cerliponase alfa. Patients receiving or expected to receive cerliponase alfa within 60 days of signing the informed consent form (ICF) may be eligible to enroll in the study, assuming all regulatory requirements for sites that have agreed to participate and protocol inclusion criteria are met. Data may be collected for all or some of the assessments as outlined in the protocol, dependent upon the clinic's and/or individual patient's standard of care.

Trial Health

75
On Track

Trial Health Score

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

Enrollment
35

participants targeted

Target at P25-P50 for all trials

Timeline
52mo left

Started Aug 2020

Longer than P75 for all trials

Geographic Reach
1 country

16 active sites

Status
active not recruiting

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

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Progress57%
Aug 2020Aug 2030

First Submitted

Initial submission to the registry

July 15, 2020

Completed
5 days until next milestone

First Posted

Study publicly available on registry

July 20, 2020

Completed
1 month until next milestone

Study Start

First participant enrolled

August 19, 2020

Completed
10 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

August 31, 2030

Expected
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

August 31, 2030

Last Updated

January 14, 2026

Status Verified

January 1, 2026

Enrollment Period

10 years

First QC Date

July 15, 2020

Last Update Submit

January 13, 2026

Conditions

Keywords

Batten's DiseaseCLN2TPP1 enzyme

Outcome Measures

Primary Outcomes (1)

  • Safety surveillance of cerliponase alfa

    To evaluate the long-term safety of cerliponase alfa in patients with neuronal ceroid lipofuscinosis Type 2 (CLN2 disease).

    10 years

Secondary Outcomes (2)

  • Hypersensitivity

    10 years

  • Severe SAE impact on patient's motor and language functions

    10 years

Study Arms (1)

Cerliponase alfa patients

Patients who are currently on or plan to start taking cerliponase alfa within 60 days of signing the study informed consent form.

Drug: Cerliponase AlfaDevice: Administration Kit

Interventions

Commercially available product provided to patient by participating clinic site.

Also known as: Brineura
Cerliponase alfa patients

Commercially available administration kit provided to the patient by participating clinic site.

Cerliponase alfa patients

Eligibility Criteria

Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64), Older Adult (65+)
Sampling MethodNon-Probability Sample
Study Population

CLN2 patients who are currently taking or intend to take cerliponase alfa within 60 days of signing the informed consent form.

You may qualify if:

  • Diagnosed with CLN2 disease.
  • Currently receiving or plan to begin treatment with cerliponase alfa.
  • Written informed consent/assent obtained.

You may not qualify if:

  • \. Currently receiving treatment in another investigational device or drug study.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (16)

Arkansas Children's Hospital

Little Rock, Arkansas, 72205, United States

Location

Children's Hospital of Orange County

Orange, California, 92868, United States

Location

Children's Hospital of Colorado

Aurora, Colorado, 80045, United States

Location

Children's National Hospital

Washington D.C., District of Columbia, 20010, United States

Location

Advent Health

Orlando, Florida, 32803, United States

Location

Children's Healthcare of Atlanta

Atlanta, Georgia, 30329, United States

Location

Rush University Medical Center

Chicago, Illinois, 60612, United States

Location

Boston Children's Hospital,

Boston, Massachusetts, 02115, United States

Location

Children's Hospital Minnesota

Minneapolis, Minnesota, 55404, United States

Location

NYU Langone Medical Center

New York, New York, 10017, United States

Location

Mt. Sinai School of Medicine

New York, New York, 10029, United States

Location

University of Rochester Medical Center

Rochester, New York, 14642, United States

Location

Nationwide Children's Hospital

Columbus, Ohio, 43130, United States

Location

University of Oklahoma Health Sciences Center

Oklahoma City, Oklahoma, 73104, United States

Location

Texas Children's Hospital

Houston, Texas, 77030, United States

Location

Seattle Children's Hospital

Seattle, Washington, 98145-5005, United States

Location

MeSH Terms

Conditions

Neuronal Ceroid-Lipofuscinoses

Interventions

cerliponase alfa

Condition Hierarchy (Ancestors)

Heredodegenerative Disorders, Nervous SystemNeurodegenerative DiseasesNervous System DiseasesGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesLipidosesLipid Metabolism, Inborn ErrorsMetabolism, Inborn ErrorsLipid Metabolism DisordersMetabolic DiseasesNutritional and Metabolic Diseases

Study Officials

  • Medical Director, MD

    BioMarin Pharmaceutical

    STUDY DIRECTOR

Study Design

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

Study Record Dates

First Submitted

July 15, 2020

First Posted

July 20, 2020

Study Start

August 19, 2020

Primary Completion (Estimated)

August 31, 2030

Study Completion (Estimated)

August 31, 2030

Last Updated

January 14, 2026

Record last verified: 2026-01

Data Sharing

IPD Sharing
Will not share

Locations