Study of Cardiac and Paroxysmal Abnormalities in Rett Syndrome

Rett Syndrome Clinical Trial

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT00004773
• Other study ID #: 199/11798
• Secondary ID: BCM-H2465
• Status: Completed
• Phase: N/A
• First received: February 24, 2000
• Last updated: June 23, 2005
• Start date: August 1995
• Est. completion date: June 1997

Study information

• Verified date: January 1997
• Source: Office of Rare Diseases (ORD)
• Contact: n/a
• Is FDA regulated: No
• Health authority: United States: Federal Government
• Study type: Observational

Clinical Trial 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.

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 information / eligibility

• Status: Completed
• Enrollment: 95
• Est. completion date: June 1997
• Accepts healthy volunteers: No
• Gender: Female
• Age group: N/A and older

Eligibility

• Classical Rett syndrome meeting Rett Syndrome Diagnostic Work Group criteria

• Age-matched girls without neurologic or cardiac problems entered as controls

Study Design

Primary Purpose: Screening

Related Conditions & MeSH terms

• Rett Syndrome

Locations

Country	Name	City	State
n/a

Sponsors (2)

Lead Sponsor	Collaborator
Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)	Baylor College of Medicine

References & Publications (3)

Glaze DG, Schultz RJ. Rett Syndrome: Meeting the Challenge of This Gender-Specific Neurodevelopmental Disorder. Medscape Womens Health. 1997 Jan;2(1):3. — View Citation

Glaze DG. Commentary: the challenge of Rett syndrome. Neuropediatrics. 1995 Apr;26(2):78-80. — View Citation

Sekul EA, Moak JP, Schultz RJ, Glaze DG, Dunn JK, Percy AK. Electrocardiographic findings in Rett syndrome: an explanation for sudden death? J Pediatr. 1994 Jul;125(1):80-2. — View Citation