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

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details. Identifier: NCT00004753
Recruitment Status : Completed
First Posted : February 25, 2000
Last Update Posted : June 24, 2005
National Institute of Neurological Disorders and Stroke (NINDS)
University of California, Los Angeles
Information provided by:
Office of Rare Diseases (ORD)

Brief Summary:

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.

Condition or disease
Huntington's Disease

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.

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Study Type : Observational
Enrollment : 90 participants
Primary Purpose: Screening
Study Start Date : August 1993

Resource links provided by the National Library of Medicine

Information from the National Library of Medicine

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Ages Eligible for Study:   Child, Adult, Older Adult
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

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its identifier (NCT number): NCT00004753

Sponsors and Collaborators
National Center for Research Resources (NCRR)
National Institute of Neurological Disorders and Stroke (NINDS)
University of California, Los Angeles
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Study Chair: John C. Mazziotta University of California, Los Angeles

Layout table for additonal information Identifier: NCT00004753     History of Changes
Other Study ID Numbers: 199/11677
First Posted: February 25, 2000    Key Record Dates
Last Update Posted: June 24, 2005
Last Verified: December 2001

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

Additional relevant MeSH terms:
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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