Study of Cardiac and Paroxysmal Abnormalities in Rett Syndrome

Descriptive Information
Brief Title † Study of Cardiac and Paroxysmal Abnormalities in Rett Syndrome
Official Title †
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.
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 Phase N/A
Study Type † Observational
Study Design † Primary Purpose: Screening
Primary Outcome Measure †
Secondary Outcome Measure †
Condition † Rett Syndrome
Intervention †
Study Arms / Comparison Groups
Publications *

* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Recruitment Information
Recruitment Status †
Estimated Enrollment † 95
Start Date † August 1995
Completion Date June 1997
Primary Completion Date
Eligibility Criteria † - Classical Rett syndrome meeting Rett Syndrome Diagnostic Work Group criteria - Age-matched girls without neurologic or cardiac problems entered as controls
Gender Female
Ages N/A - N/A
Accepts Healthy Volunteers No
Contacts ††
Location Countries †
Administrative Information
NCT ID † NCT00004773
Organization ID 199/11798
Secondary IDs †† BCM-H2465
Responsible Party
Study Sponsor † Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)
Collaborators †† Baylor College of Medicine
Investigators † Study Chair: Daniel G. Glaze, Baylor College of Medicine
Information Provided By
Verification Date January 1997
First Received Date † February 24, 2000
Last Updated Date June 23, 2005
† Required WHO trial registration data element.
†† WHO trial registration data element that is required only if it exists.
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