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Long-Term Study of Cerebral Glucose Metabolism in Huntington's Disease

This study has been completed.
National Institute of Neurological Disorders and Stroke (NINDS)
University of California, Los Angeles
Information provided by:
Office of Rare Diseases (ORD) Identifier:
First received: February 24, 2000
Last updated: June 23, 2005
Last verified: December 2001

OBJECTIVES: I. Correlate clinical outcome with cerebral glucose metabolism in patients with Huntington's disease (HD) and their at-risk relatives.

II. Evaluate the efficacy of cerebral glucose metabolism in observing the pathophysiologic development of HD, monitoring responses to experimental therapy, and predicting HD genotype.

III. Identify, define, and describe the natural history of pathophysiologic lesions in HD.

IV. Characterize the genotypic and phenotypic expression of the HD gene.

Huntington's Disease

Study Type: Observational
Study Design: Primary Purpose: Screening

Resource links provided by NLM:

Further study details as provided by Office of Rare Diseases (ORD):

Estimated Enrollment: 90
Study Start Date: August 1993
Detailed Description:

PROTOCOL OUTLINE: Participants are screened for Huntington's disease, including cerebral glucose metabolism assessment and genetic testing.

Studies include a detailed family history and neurologic, psychometric, and neurobehavioral evaluations. Imaging includes positron emission tomography with fluorodeoxyglucose and brain magnetic resonance imaging.

A genotype assessment is performed; genetic results are not disclosed to patients or relatives.


Ages Eligible for Study:   Child, Adult, Senior
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   Yes


--Disease Characteristics--

  • Documented family history of Huntington's disease (HD)
  • Symptomatic HD: chorea required
  • At-risk for HD: no detectable systemic or oculomotor abnormality
  • Age-matched control subjects
  • No history of inherited neurological disease
  • No general or neurologic abnormality

--Prior/Concurrent Therapy--

At least 4 weeks since other HD treatment, e.g.:

  • Haloperidol
  • Benzodiazepine
  • Other tranquilizers or neuroleptics

--Patient Characteristics--

  • No pregnant women
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its identifier: NCT00004753

Sponsors and Collaborators
National Center for Research Resources (NCRR)
National Institute of Neurological Disorders and Stroke (NINDS)
University of California, Los Angeles
Study Chair: John C. Mazziotta University of California, Los Angeles
  More Information Identifier: NCT00004753     History of Changes
Other Study ID Numbers: 199/11677
Study First Received: February 24, 2000
Last Updated: June 23, 2005

Keywords provided by Office of Rare Diseases (ORD):
Huntington's disease
neurologic and psychiatric disorders
rare disease

Additional relevant MeSH terms:
Huntington Disease
Basal Ganglia Diseases
Brain Diseases
Central Nervous System Diseases
Nervous System Diseases
Movement Disorders
Heredodegenerative Disorders, Nervous System
Neurodegenerative Diseases
Genetic Diseases, Inborn
Cognition Disorders
Neurocognitive Disorders
Mental Disorders processed this record on July 19, 2017