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Physiopathologic protocols

Sleep evaluation in the childhood type of myotonic dystrophy

Investigator
Dr D. Héron
Sponsor
none
Patient numbers
Total
At the Institute of Myology
 
40
40
Projected dates
Beginning
Ending
 
May 2004
December 2006
Financing
AFM
Summary
 
The infantile form of myotonic dystrophy is caracterised by slowness, fatigue and learning difficulties at school. Hypersomnia is a prevalent complaint reporfed by parents and practicians in the children with the infantile form of DM.
Hypersomnia in adults with DM is well documented but no evaluation has been already performed in children.
This 2 years study will include 40 children and teenagers with the infantile form of DM, from 6 to 19 years old, who have already had a neuropsychological evaluation at the Institute of Myology.
Sleep evaluation protocol consists of :
- Self evaluation of excessive sleepiness using the Epworth sleepiness scale adapted to children
- Actimetry: evaluation of extend and speed of motion using a wrist worn activity monitoring system.
- 24 hour ambulatory polysomnography
- Multiple Sleep Latency test
These investigations will be carried out at the Sleep Laboratory of the Garches Hospital ( Dr Quera Selva )
The aim of this project is to identify the causes and to caracterise the sleep disorders in the infantile form of DM, Are sleep problems involved in the learning difficulties of these children ?
A more advanced knowledge of the problem could help to performe adapted therapeutical trials as for the adult form of DM.

 
Institut de Myologie - 47/83 Bd de l'hôpital - 75013 Paris - Tél. : +33 1 42 16 58 58