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Functional Evaluation in Patients With Urea Cycle Disorders (UCD) During a Driving Task

29 juillet 2026 mis à jour par: St. Jude Children's Research Hospital

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

This study is being done to understand how the brain works while people with urea cycle disorder (UCD) perform driving tasks that range from easy to difficult and to look at how that compares to traditional tests of thinking and attention.

Aperçu de l'étude

Statut

Recrutement

Les conditions

Trouble du cycle de l'urée

Description détaillée

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.

Type d'étude

Observationnel

Inscription (Estimé)

Contacts et emplacements

Cette section fournit les coordonnées de ceux qui mènent l'étude et des informations sur le lieu où cette étude est menée.

Coordonnées de l'étude

Nom: Jean Laboe, RN
Numéro de téléphone: 901-595-1693
E-mail: jean.laboe@stjude.org

Lieux d'étude

États-Unis
- Tennessee
  - Memphis, Tennessee, États-Unis, 38105
    - Recrutement
    - St. Jude Children's Research Hospital
    - Contact:
      
      Jean Laboe, RN
      
      Numéro de téléphone: 901-595-1693
      
      E-mail: jean.laboe@stjude.org

Critères de participation

Les chercheurs recherchent des personnes qui correspondent à une certaine description, appelée critères d'éligibilité. Certains exemples de ces critères sont l'état de santé général d'une personne ou des traitements antérieurs.

Critère d'éligibilité

Âges éligibles pour étudier

Enfant
Adulte

Accepte les volontaires sains

Oui

Méthode d'échantillonnage

Échantillon non probabiliste

Population étudiée

Individuals who meet the Eligibility Criteria and agree to participate.

La description

Inclusion Criteria:

Individuals 16 to 40 years with molecular or biochemical confirmation of a urea cycle disorder.
Healthy individuals will serve as an age and sex matched comparison group. Active driver's license for at least 6 months
Read and understand English. as the tasks have not been validated in other languages
IQ >=70.

Exclusion Criteria:

Skin disease that affects the scalp
Past or present vascular disease
Head trauma with loss of consciousness in the last year or evidence of cognitive impairment due to and persisting after head trauma
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
The participant is taking a stimulant or antidepressant
Allergy to any component of the device, although expected to be rare
History of psychosis
Self-reported motion or cyber sickness
Self-reported illicit substance use
Vision disorder

Plan d'étude

Cette section fournit des détails sur le plan d'étude, y compris la façon dont l'étude est conçue et ce que l'étude mesure.

Comment l'étude est-elle conçue ?

Détails de conception

Nombre de groupes / cohortes

Cohortes et interventions

Groupe / Cohorte
UCD group Participants with urea cycle disorders (UCD) who meet the Eligibility Criteria and consent to participate
Control group Healthy controls who meet the Eligibility Criteria and consent to participate

Que mesure l'étude ?

Principaux critères de jugement

Mesure des résultats	Description de la mesure	Délai
Changes in Oxyhemoglobin (HbO), Deoxyhemoglobin (HbR) and Total Hemoglobin (HbT) concentrations in the right and left prefrontal cortex in participants with urea cycle disorders (UCD) compared to healthy control volunteers Délai: Day 1, during driving simulator task	Investigators will compare prefrontal cortex hemoglobin concentrations during low cognitive load between patients with UCD and healthy age and sex matched control participants using functional near-infrared spectroscopy (fNIRS). Mean values of hemoglobin concentration differences on the right and left prefrontal cortex between individuals, with and without UCD, during the low cognitive load task will be calculated and compared using two sample t-tests (for normally distributed data) or Wilcoxon rank sum tests (for nonnormally distributed data). P values will be adjusted for multiple comparisons using the Bonferroni method.	Day 1, during driving simulator task

Mesures de résultats secondaires

Mesure des résultats	Description de la mesure	Délai
Changes in HbO, HbR and HbT concentrations in the left and right parietal and occipital brain regions in participants with urea cycle disorders (UCD) compared to healthy control volunteers Délai: Day 1, during driving simulator task	Investigators will compare left and right parietal and occipital regions hemoglobin concentrations during low cognitive load between patients with UCD and healthy age and sex matched control participants using functional near-infrared spectroscopy (fNIRS). Mean values of hemoglobin concentration differences on the left and right parietal and occipital regions between individuals, with and without UCD, during the low cognitive load task will be calculated and compared using two sample t-tests (for normally distributed data) or Wilcoxon rank sum tests (for nonnormally distributed data). P values will be adjusted for multiple comparisons using the Bonferroni method.	Day 1, during driving simulator task

Collaborateurs et enquêteurs

C'est ici que vous trouverez les personnes et les organisations impliquées dans cette étude.

Parrainer

St. Jude Children's Research Hospital

Les enquêteurs

Chercheur principal: Andrea Gropman, M.D., St. Jude Children's Research Hospital

Publications et liens utiles

La personne responsable de la saisie des informations sur l'étude fournit volontairement ces publications. Il peut s'agir de tout ce qui concerne l'étude.

Liens utiles

Dates d'enregistrement des études

Ces dates suivent la progression des dossiers d'étude et des soumissions de résultats sommaires à ClinicalTrials.gov. Les dossiers d'étude et les résultats rapportés sont examinés par la Bibliothèque nationale de médecine (NLM) pour s'assurer qu'ils répondent à des normes de contrôle de qualité spécifiques avant d'être publiés sur le site Web public.

Dates principales de l'étude

Début de l'étude (Réel)

20 juillet 2026

Achèvement primaire (Estimé)

1 août 2031

Achèvement de l'étude (Estimé)

1 août 2032

Dates d'inscription aux études

Première soumission

8 juillet 2026

Première soumission répondant aux critères de contrôle qualité