Study of Cardiac and Paroxysmal Abnormalities in Rett Syndrome

Brief Title

Study of Cardiac and Paroxysmal Abnormalities in Rett Syndrome

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 Type

Observational

Condition

Rett Syndrome

Publications

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

Recruitment Information

Estimated Enrollment

Start Date

August 1995

Completion Date

June 1997

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

Daniel G. Glaze, ,

Administrative Informations

NCT ID

NCT00004773

Organization ID

199/11798

Secondary IDs

BCM-H2465

Study Sponsor

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

Collaborators

 Baylor College of Medicine

Study Sponsor

Daniel G. Glaze, Study Chair, Baylor College of Medicine

Verification Date

January 1997