Natural History Study of Infants and Children With Developmental and Epileptic Encephalopathies

NCT ID: NCT04537832

Last Updated: 2023-06-12

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

Total Enrollment

58 participants

Study Classification

OBSERVATIONAL

Study Start Date

2021-01-18

Study Completion Date

2023-03-31

Brief Summary

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This is a multicenter, prospective, 2-year observational study in infants and children with developmental and epileptic encephalopathies (DEEs). The DEE currently being investigated is SCN1A-positive Dravet Syndrome.

Detailed Description

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This prospective, longitudinal, natural history master protocol has been designed to define the seizure, neurodevelopmental, and behavioral characteristics of SCN1A-positive Dravet Syndrome in infants and children between 6 and 60 months. It will also explore the impact of the disease on the participant's parent/caregiver quality of life (QoL) and healthcare resource utilization (HCRU).

Conditions

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Dravet Syndrome

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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SCN1A-positive Dravet Syndrome

Participants aged between 6 and 60 months of age who have SCN1A-positive Dravet Syndrome. Clinical, neurocognitive, laboratory, the burden of disease, and health care resource utilization will be assessed.

No Intervention

Intervention Type OTHER

No Intervention

Interventions

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No Intervention

No Intervention

Intervention Type OTHER

Eligibility Criteria

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Inclusion Criteria

* Aged between 6 months and 60 months.
* Confirmed SCN1A mutation.
* Normal development prior to onset of first seizure as defined by the Centers for Disease -Control and Prevention (CDC 2019).
* Onset of seizures between age 3 and 15 months, inclusive.

Exclusion Criteria

* Copy number variant of SCN1A, including SCN1A microdeletion, if affecting other genes.
* SCN1A mutation present on both alleles.
* Known pathogenic or clinically suspected mutation in a seizure-associated gene besides SCN1A.
* Confirmed mutation in a gene besides SCN1A that is known to increase the severity of the seizure phenotype.
* Known gain-of-function genetic mutation, as defined by functional studies, including p.Thr226Met.
* History of notable developmental deficit that was evident prior to seizure onset.
* Known central nervous system structural abnormality as found on magnetic resonance imaging or computed tomography scan of brain.
* Currently taking or has taken for 6 or more consecutive weeks anti-seizure medications (ASMs) at a therapeutic dose that are contraindicated in SCN1A-positive Dravet Syndrome, including sodium channel blockers.
* Known concomitant genetic mutation or clinical comorbidity that potentially confounds typical Dravet phenotype.
Minimum Eligible Age

6 Months

Maximum Eligible Age

60 Months

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Encoded Therapeutics

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Salvador Rico, M.D., Ph.D

Role: STUDY_DIRECTOR

Encoded Therapeutics

Locations

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Children's Hospital Los Angeles

Los Angeles, California, United States

Site Status

UCSF Benioff Children's Hospital

San Francisco, California, United States

Site Status

Children's Hospital Colorado

Aurora, Colorado, United States

Site Status

Nicklaus Children's Hospital

Miami, Florida, United States

Site Status

Ann and Robert H. Lurie Children's Hospital of Chicago

Chicago, Illinois, United States

Site Status

Northeast Regional Epilepsy Group

Hackensack, New Jersey, United States

Site Status

Abigail Wexner Research Institute at Nationwide Children's Hospital

Columbus, Ohio, United States

Site Status

Oregon Health and Science University

Portland, Oregon, United States

Site Status

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status

Le Bonheur Children's Hospital

Memphis, Tennessee, United States

Site Status

Cook Children's Medical Center

Fort Worth, Texas, United States

Site Status

Multicare Institute for Research and Innovation

Tacoma, Washington, United States

Site Status

Austin Hospital - Melbourne Brain Centre

Heidelberg, Victoria, Australia

Site Status

Hospital de la Santa Creu i Sant Pau

Barcelona, , Spain

Site Status

Hospital Universitari i Politècnic La Fe

Valencia, , Spain

Site Status

Queen Elizabeth Hospital

Glasgow, , United Kingdom

Site Status

Countries

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United States Australia Spain United Kingdom

Other Identifiers

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ETX-DS-001

Identifier Type: -

Identifier Source: org_study_id

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