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

University Hospital, Lille

Status: Completed

Conditions: Myotonic Dystrophy, Steinert

Study type: Observational

Funder types: Other

Identifiers

NCT03764150

2009-A00588-49 (Other Identifier)

2009_04/0923

Details and patient eligibility

About

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.

Enrollment: 113 patients

Sex: All

Ages: 18+ years old

Volunteers: No Healthy Volunteers

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

Trial design

113 participants in 2 patient groups

PaCO2> 45 mmHg

Description:

Diurnal hypercapnia defined by PaCO2\> 45 mmHg

PaCO2 <45 mmHg

Description:

PaCO2 diurnal within the limits of normal (PaCO2 \<45 mmHg)