Actively Recruiting

Age: 0 - 18Years
All Genders
Healthy Volunteers
ID05651204

Electrophysiological Biomarkers of GABA Metabolism in Children With SCN1A+ Dravet Syndrome

Led by Cook Children's Health Care System · Updated on 2022-12-14

36

Participants Needed

1

Research Sites

156 weeks

Total Duration

On this page

Sponsors

C

Cook Children's Health Care System

Lead Sponsor

E

Encoded Therapeutics

Collaborating Sponsor

AI-Summary

What this Trial Is About

Researchers are investigating the levels of GABA, a neurotransmitter involved in neuron excitation and inhibition, in children with SCN1A-positive Dravet Syndrome (DS) and neurodeveloping children. The study focuses on understanding abnormal GABA mechanisms in DS by comparing brain activity and blood GABA levels. This research aims to develop noninvasive biomarkers to monitor brain GABA levels, which may help guide future treatments targeting GABA regulation in children with DS. The study involves measuring GABA levels in the brain and blood using noninvasive techniques. Blood samples will be collected by a trained phlebotomist following hospital procedures. Brain activity will be recorded through various methods including BOLD MRI, MEG, HD-EEG, and TMS to assess cortical responses related to GABA. Participants include children with SCN1A+ DS and age-matched healthy controls. Participants will undergo blood collection and multiple brain imaging and electrophysiological assessments lasting from 30 minutes up to 3 hours depending on the test. The study evaluates correlations between blood GABA levels, brain activity patterns, and MRI responses. Researchers will monitor these outcomes to better understand GABA metabolism in DS. The study is designed for children up to 18 years old and involves no treatment intervention, focusing on observation and diagnostic testing.

CONDITIONS

Brief Title

GABA Biomarkers in Dravet Syndrome

Who Can Participate

Age: 0 - 18Years
All Genders
Healthy Volunteers

Eligibility Criteria

Eligible

You may qualify if you...

  • Authorized representative (parent/caregiver) must provide informed consent; participant capable of assent must provide it
  • Participant and caregiver willing and able to comply with study requirements
  • Participant aged 0 months to 18 years at consent
  • Confirmed pathogenic or likely pathogenic SCN1A mutation by genetic testing
  • Normal development before first seizure as defined by CDC 2019
  • Seizure onset between 3 and 5 months of age
  • Diagnosis of Dravet Syndrome by a pediatric neurologist
Not Eligible

You will not qualify if you...

  • Presence of SCN1A copy number variant affecting other genes
  • SCN1A mutation on both alleles
  • Pathogenic or suspected mutation in seizure gene other than SCN1A
  • Mutation in gene increasing seizure severity besides SCN1A
  • Known gain-of-function mutation including p.Thr226Met
  • Notable developmental deficit before seizure onset
  • Known central nervous system structural abnormality inconsistent with DS
  • Metal implants
  • Baclofen pump
  • Inability or unwillingness to provide informed consent or assent

AI-Screening

AI-Powered Screening

Complete this quick 3-step screening to check your eligibility

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Your Study Journey

Screening

Duration - 2 to 4 weeks

Participants are screened for eligibility to participate in the trial.

1 visit (in-person)

Diagnostic Evaluation

Duration - Up to 3 hours

Participants undergo blood tests and non-invasive electrophysiological procedures to assess GABA levels and brain activity.

1 visit (in-person)

Long-term Monitoring

Duration - Up to several years until study completion

Participants are observed for further understanding of GABA metabolism in Dravet Syndrome without receiving treatment.

Visit schedule varies depending on assessments

Trial Site Locations

Total: 1 location

1

Cook Children's Medical Center

Fort Worth, Texas, United States, 76104

Actively Recruiting

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Research Team

S

Sabrina Shandley, PhD

How is the study designed?

Study Type

OBSERVATIONAL

Masking

N/A

Allocation

N/A

Model

N/A

Primary Purpose

N/A

Number of Arms

2

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