Factors Associated With Hypoventilation in the Myotonic Dystrophy, Progressive Profile Over 5 Years

Myotonic Dystrophy, Steinert Clinical Trial

— Steinert  

Official title:

Factors Associated With Hypoventilation in the Myotonic Dystrophy, Progressive Profile Over 5 Years

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT03764150
• Other study ID #: 2009_04/0923
• Secondary ID: 2009-A00588-49
• Status: Recruiting
• Start date: June 30, 2010
• Est. completion date: September 2023

Study information

• Verified date: January 2021
• Source: University Hospital, Lille
• Contact: Thierry Perez, MD
• Email: thierry.perez[@]chru-lille.fr
• Is FDA regulated: No
• Study type: Observational

Clinical Trial Summary

The aim of this study is to determine the factors associated with alveolar hypoventilation in terms of cognitive impairment, daytime sleepiness, respiratory function, nocturnal respiratory events This evaluation will clarify the clinical phenotypes of respiratory disease in myotonic steinert dystrophy.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 160
• Est. completion date: September 2023
• Est. primary completion date: September 2023
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

Inclusion Criteria:

• Myotonic dystrophy of Steinert genetically confirmed (number of known CTG repeats), in its classical form of adult

Exclusion Criteria:

• Inability to perform EFR assessment or polysomnography

• Moderate to severe asthma according to the GINA classification

• moderate to severe COPD (> stage 1 of the GOLD recommendations)

• Other significant respiratory pathology

• Patient already on NIV, CPAP or under oxygen therapy

• Impossibility of receiving enlightened information

• Inability to participate in the entire study

Related Conditions & MeSH terms

• Hypoventilation
• Myotonic Dystrophy
• Myotonic Dystrophy, Steinert

Locations

Country	Name	City	State
France	Centre Hospitalier Regional D' Angers	Angers
France	Ap-Hp Hu Paris Site Raymond Poincare	Garches
France	Hôpital Calmette, CHRU	Lille
France	Chu Montpellier	Montpellier
France	Chu de Nantes :Site Hôtel-Dieu- Hme - Nantes 1	Nantes
France	Ap-Hp Hu Pitie Salpetriere	Paris

Sponsors (1)

Lead Sponsor	Collaborator
University Hospital, Lille

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Change of measurement of spirometry	composite predictive factors of alveolar hypoventilation	every year during 5 years
Primary	Change respiratory force	composite predictive factors of alveolar hypoventilation to change Inspiratory and expiratory force	every year during 5 years
Primary	Change CO2 response (Read test)	composite predictive factors of alveolar hypoventilation	every year during 5 years
Primary	Change subjective sleepiness (Epworth)	composite predictive factors of alveolar hypoventilation	every year during 5 years
Primary	change Krupp's Fatigue Severity Scale (KFSS)	composite predictive factors of alveolar hypoventilation This is a short questionnaire of 9 questions to which the patient responds on a Likert scale ranging from 1 to 7	every year during 5 years
Primary	change cognitive impairment by Trail Making Test A & B	composite predictive factors of alveolar hypoventilation Trail Making Test is a neuropsychological test of visual attention and task switching. It consists of two parts in which the subject is instructed to connect a set of 25 dots as quickly as possible while still maintaining accuracy.	every year during 5 years
Primary	change Oximetry	composite predictive factors of alveolar hypoventilation	every year during 5 years