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.
Study Type
OBSERVATIONAL
Enrollment
113
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
Change of measurement of spirometry
composite predictive factors of alveolar hypoventilation
Time frame: every year during 5 years
Change respiratory force
composite predictive factors of alveolar hypoventilation to change Inspiratory and expiratory force
Time frame: every year during 5 years
Change CO2 response (Read test)
composite predictive factors of alveolar hypoventilation
Time frame: every year during 5 years
Change subjective sleepiness (Epworth)
composite predictive factors of alveolar hypoventilation
Time frame: every year during 5 years
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
Time frame: every year during 5 years
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.
Time frame: every year during 5 years
change Oximetry
composite predictive factors of alveolar hypoventilation
Time frame: every year during 5 years
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Chu de Nantes :Site Hôtel-Dieu- Hme - Nantes 1
Nantes, France
Ap-Hp Hu Pitie Salpetriere
Paris, France