Recruiting

Cognitive and Motor Function Examination in Patients With Urea Cycle Disorders (UCD) During a Driving Task Using Functional Near Infrared Spectroscopy (fNIRS)

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What is being collected

Data Collection

Collected from today forward - Prospective
Who is being recruted

Amino Acid Metabolism, Inborn Errors+9

+ Brain Diseases

+ Brain Diseases, Metabolic

From 16 to 40 Years
+15 Eligibility Criteria
See all eligibility criteria
How is the trial designed

Case-Control

Comparing exposures between individuals with and without disease in order to identify potential risk factors.
Observational
Study Start: July 2026
See protocol details

Summary

Principal SponsorSt. Jude Children's Research Hospital
Study ContactJean Laboe, RNMore contacts
Last updated: July 14, 2026
Sourced from a government-validated database.Claim as a partner

Study start date: July 15, 2026

Actual date on which the first participant was enrolled.

Primary Objective * To use fNIRS to study brain functional behavior in subjects with UCD by measuring the changes in HbO, HbR and HbT concentrations versus healthy control volunteers during the baseline driving task on the left and right prefrontal cortex (LPFC, RPFC). Secondary Objective * To include other cortical regions such as temporal and occipital regions, to investigate the neurocognitive changes in these regions which may or may not change with driving. Thus, we will measure changes in HbO, HbR and HbT concentrations during the baseline driving task in four more brain regions (left and right parietal and occipital regions, LPA, RPA, LOL, ROL). This is a non-therapeutic, non-interventional study in which brain fNIRS (functional near-infrared spectroscopy) measures will be compared under simulated driving conditions between patients with UCD vs. age and sex-matched control participants. Participants who agree to participate will have Information about their medical history collected, have a basic neurological exam and answer questions about their health, diet, and driving habits. They will complete some simple thinking and memory tests. These tests will help investigators understand skills like memory, attention, and problem-solving. Participants will also wear a soft cap with small sensors called an fNIRS (functional near-infrared spectroscopy) device while they complete a driving simulator activity. This cap is noninvasive and measures how the brain uses oxygen while they drive in the simulator. All study activities will be completed on the day of enrollment.

Principal SponsorSt. Jude Children's Research Hospital
Study ContactJean Laboe, RNMore contacts
Last updated: July 14, 2026
Sourced from a government-validated database.Claim as a partner

Protocol

This section provides details of the study plan, including how the study is designed and what the study is measuring.
Design Details

60 patients to be enrolled

Total number of participants that the clinical trial aims to recruit.

Case-Control

These studies compare people who have a disease (cases) with those who don't (controls). The goal is to look back at previous exposures or risk factors to identify what might have contributed to the disease.

Eligibility

Researchers look for people who fit a certain description, called eligibility criteria: person's general health condition or prior treatments.
Conditions
Criteria

Any sex

Biological sex of participants that are eligible to enroll.

From 16 to 40 Years

Range of ages for which participants are eligible to join.

Healthy volunteers allowed

If individuals who are healthy and do not have the condition being studied can participate.

Conditions

Pathology

Amino Acid Metabolism, Inborn ErrorsBrain DiseasesBrain Diseases, MetabolicCentral Nervous System DiseasesMetabolic DiseasesMetabolism, Inborn ErrorsCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesNervous System DiseasesNutritional and Metabolic DiseasesBrain Diseases, Metabolic, InbornGenetic Diseases, InbornUrea Cycle Disorders, Inborn

Criteria

4 inclusion criteria required to participate
Individuals 16 to 40 years with molecular or biochemical confirmation of a urea cycle disorder

Read and understand English. as the tasks have not been validated in other languages

IQ >= 70

Healthy individuals will serve as an age and sex matched comparison group. Active driver's license for at least 6 months

11 exclusion criteria prevent from participating
Skin disease that affects the scalp

Allergy to any component of the device, although expected to be rare

Motor movement disorder which may cause excessive movement or impede use of the steering wheel and/or pedal

Premature birth or birth before 32 weeks of gestation

Show More Criteria

Study Plan

Find out more about all the medication administered in this study, their detailed description and what they involve.
Treatment Groups
Study Objectives

2 intervention groups are designated in this study

This study does not include a placebo group 

Treatment Groups

Study Objectives

Primary Objectives

Secondary Objectives

Study Centers

These are the hospitals, clinics, or research facilities where the trial is being conducted. You can find the location closest to you and its status.

This study has 1 location

Recruiting

St. Jude Children's Research Hospital

Memphis, United StatesOpen St. Jude Children's Research Hospital in Google Maps
Recruiting
One Study Center