Study of Cardiac and Paroxysmal Abnormalities in Rett Syndrome

NCT ID: NCT00004773

Last Updated: 2005-06-24

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Total Enrollment

95 participants

Study Classification

OBSERVATIONAL

Study Start Date

1995-08-31

Study Completion Date

1997-06-30

Brief Summary

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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

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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.

Conditions

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Rett Syndrome

Keywords

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Rett syndrome neurologic and psychiatric disorders rare disease

Eligibility Criteria

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Inclusion Criteria

* Classical Rett syndrome meeting Rett Syndrome Diagnostic Work Group criteria
* Age-matched girls without neurologic or cardiac problems entered as controls
Minimum Eligible Age

0 Years

Eligible Sex

FEMALE

Accepts Healthy Volunteers

No

Sponsors

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Baylor College of Medicine

OTHER

Sponsor Role collaborator

Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)

NIH

Sponsor Role lead

Principal Investigators

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Daniel G. Glaze

Role: STUDY_CHAIR

Baylor College of Medicine

References

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Glaze DG. Commentary: the challenge of Rett syndrome. Neuropediatrics. 1995 Apr;26(2):78-80. doi: 10.1055/s-2007-979728. No abstract available.

Reference Type BACKGROUND
PMID: 7566458 (View on PubMed)

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

Reference Type BACKGROUND
PMID: 9746669 (View on PubMed)

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. doi: 10.1016/s0022-3476(94)70128-8.

Reference Type BACKGROUND
PMID: 8021793 (View on PubMed)

Other Identifiers

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BCM-H2465

Identifier Type: -

Identifier Source: secondary_id

199/11798

Identifier Type: -

Identifier Source: org_study_id