Trial record 37 of 72 for:    rett

Study of Cardiac and Paroxysmal Abnormalities in Rett Syndrome

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: NCT00004773
Recruitment Status : Completed
First Posted : February 25, 2000
Last Update Posted : June 24, 2005
Baylor College of Medicine
Information provided by:
Office of Rare Diseases (ORD)

Brief Summary:

OBJECTIVES: I. Evaluate electrocardiographic parameters, including QT and PR intervals and QRS morphology/duration, across clinical stages in patients with Rett syndrome.

II. Characterize abnormalities of cardiac conduction and repolarization. III. Assess arrhythmias, heart rate variability, and autonomic nervous system function in these patients using 24-hour Holter monitoring.

IV. Record events believed to represent seizures with video, electroencephalogram (EEG), and polygraph monitoring in patients who have more than 1 clinical seizure every 5 days.

V. Characterize these events with respect to clinical manifestations, EEG correlates, and other physiologic data.

VI. Determine the frequency of seizures vs. events without electrographic correlates in these patients.

VII. Determine whether Rett syndrome patients have characteristic or unique types of seizures and/or an epileptic syndrome.

Condition or disease
Rett Syndrome

Detailed Description:

PROTOCOL OUTLINE: Patients receive an electrocardiogram at baseline and every 6 months for 3 years. Clinical stage II patients undergo 24-hour Holter monitoring on the same schedule; age-matched controls are tested once.

A group of patients with more than 1 seizure or possible seizure every 5 days undergo 5-day continuous electroencephalogram/polygraphic/video monitoring, with respiratory effort assessment, end tidal carbon dioxide and oxygen saturation levels, and a seizure log.

Study Type : Observational
Enrollment : 95 participants
Primary Purpose: Screening
Study Start Date : August 1995
Study Completion Date : June 1997

Resource links provided by the National Library of Medicine

MedlinePlus related topics: Rett Syndrome

Information from the National Library of Medicine

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Ages Eligible for Study:   Child, Adult, Older Adult
Sexes Eligible for Study:   Female
Accepts Healthy Volunteers:   No
  • Classical Rett syndrome meeting Rett Syndrome Diagnostic Work Group criteria
  • Age-matched girls without neurologic or cardiac problems entered as controls

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): NCT00004773

Sponsors and Collaborators
Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)
Baylor College of Medicine
Study Chair: Daniel G. Glaze Baylor College of Medicine

Publications: Identifier: NCT00004773     History of Changes
Other Study ID Numbers: 199/11798
First Posted: February 25, 2000    Key Record Dates
Last Update Posted: June 24, 2005
Last Verified: January 1997

Keywords provided by Office of Rare Diseases (ORD):
Rett syndrome
neurologic and psychiatric disorders
rare disease

Additional relevant MeSH terms:
Rett Syndrome
Pathologic Processes
Mental Retardation, X-Linked
Intellectual Disability
Neurobehavioral Manifestations
Neurologic Manifestations
Nervous System Diseases
Genetic Diseases, X-Linked
Genetic Diseases, Inborn
Heredodegenerative Disorders, Nervous System