Proof of Concept of an Anaplerotic Study Using Brain Phosphorus Magnetic Resonance Spectroscopy in Huntington Disease

The purpose of this project is to study the efficacy of an anaplerotic treatment on brain energy profile evolution at an early stage of the Huntington disease.

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Key information

Conditions

Huntington Disease Basal Ganglia Diseases Brain Diseases Central Nervous System Diseases Chorea Cognition Disorders Congenital, Hereditary, and Neonatal Diseases and Abnormalities Dementia Dyskinesias Genetic Diseases, Inborn Heredodegenerative Disorders, Nervous System Mental Disorders Movement Disorders Nervous System Diseases Neurocognitive Disorders Neurodegenerative Diseases

Age range

18 year and older

Sex eligibility

All sexes

Study type

Interventional

Phase

Phase 2

Primary location

Brain and Spine Institute

Paris, 75013, France

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

5 < UHDRS < 50
Age > 18 years
Ability to undergo MR scanning
Covered by french social security

Exclusion criteria

Evidence of psychiatric disorder
Attendant neurological disorder
Contraindications to MRI (claustrophobia, metallic or material implants)
Severe head injury
Unable to understand the protocol
Pregnancy
Failure to give informed consent
Subjects with exclusion criteria required by french law (e.g. subjects who require a legally authorized representative to obtain consent)
Unwillingness to be informed in case of abnormal MRI

Treatment and study plan

Triheptanoin 1g/kg/day

Drug

Primary outcomes

Ratio of Inorganic Phosphate (Pi) Over Phosphocreatine (PCr): Pi/PCr

Time frame: visit 1 (baseline), visit 2 (after 1 month of treatment)

The Pi/PCr Ratio is a measure of brain metabolism and it is an index of mitochondrial oxidative regulation.

A 6-cm 31P transmit/receive surface coil (RAPID Biomedical GmbH, Rimpar, Germany) was used to collect free induction decays for 4 minutes at rest, 8 minutes during visual activation with 6-Hz red/black checkerboard flashes, and 8 minutes after stimulation. Subjects were able to focus on the flashes with a nonmagnetic mirror mounted above their eyes while all lights in the room were turned off. The Pi/PCr ratio was then calculated to determine brain response to cortical activation.

Sponsors and collaborators

Lead sponsor

Institut National de la Santé Et de la Recherche Médicale, France

Other Gov

Registry information

Official study title

ETUDE PREUVE DE CONCEPT D'UNE APPROCHE ANAPLEROTIQUE UTILISANT LA SPECTROSCOPIE RMN CEREBRALE AU PHOSPHORE-31 DANS LA MALADIE DE HUNTINGTON

Acronym: TRIHEP2

Important dates

Study start: 2013
Primary completion: 2013
Study completion: 2013
First posted: Jun 20, 2013
Registry last updated: Jun 11, 2026

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

Published trials that share one or more normalized conditions with this study.

Completed