Study of Cardiac and Paroxysmal Abnormalities in Rett Syndrome
- Conditions
- Rett Syndrome
- Registration Number
- NCT00004773
- Lead Sponsor
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)
- 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.
Recruitment & Eligibility
- Status
- COMPLETED
- Sex
- Female
- Target Recruitment
- 95
Not provided
Not provided
Study & Design
- Study Type
- OBSERVATIONAL
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method
- Secondary Outcome Measures
Name Time Method