Myotonic dystrophy is an inherited muscular dystrophy causing muscle weakness and wasting. Many people with myotonic dystrophy complain about excessive daytime sleepiness. This symptom is related to disordered central respiratory control. Psychostimulants are drugs that increase alertness and include caffeine, amphetamine, selegiline, methylphenidate and modafinil. Few randomised controlled trials have evaluated the efficacy and safety of psychostimulants in myotonic dystrophy. One randomised controlled trial of selegiline involving 11 participants did not demonstrate any benefit. Two studies of another drug modafinil, one involving 40 participants, the other 20 participants, suggested inconsistent and slight benefits but they did not use the gold standard test to evaluate hypersomnia. In these two studies this drug seemed well tolerated. Further randomised trials are needed to determine the utility of psychostimulants for myotonic dystrophy.

This is a Cochrane review abstract and plain language summary, prepared and maintained by The Cochrane Collaboration, currently published in The Cochrane Database of Systematic Reviews 2010 Issue 7, Copyright © 2010 The Cochrane Collaboration. Published by John Wiley and Sons, Ltd.. The full text of the review is available in The Cochrane Library (ISSN 1464-780X).
This record should be cited as: Annane D, Moore DH, Barnes PRJ, Miller RG. Psychostimulants for hypersomnia (excessive daytime sleepiness) in myotonic dystrophy. Cochrane Database of Systematic Reviews 2006, Issue 3. Art. No.: CD003218. DOI: 10.1002/14651858.CD003218.pub2

Editorial Group: Neuromuscular Disease Group

This version first published online: October 21. 2002
Last assessed as up-to-date: April 30. 2006

Abstract

Background

Excessive daytime sleepiness is a common symptom of myotonic dystrophy. Psychostimulants are drugs increasingly used to treat hypersomnia in myotonic dystrophy.

Objectives

To search systematically for, and combine all evidence from, randomised trials relating to the effects of psychostimulants in myotonic dystrophy patients with hypersomnia.

Search strategy

We searched the Cochrane Neuromuscular Disease Trials Register (January 2006), MEDLINE (from January 1966 to January 2006) and EMBASE (from January 1980 to January 2006) for randomised trials concerning psychostimulants in myotonic dystrophy, checked the bibliographies of identified papers and made enquiries of the authors of the papers. The search for relevant studies was updated in January 2006.

Selection criteria

We considered all randomised or quasi randomised trials that have evaluated any type of psychostimulants (versus a placebo or no treatment) in children or adults with proven myotonic dystrophy and hypersomnia.

Data collection and analysis

Potentially relevant papers were scrutinised by two authors and the selection of eligible studies was agreed by them and a third author. Data were extracted by one author and checked by a second author.

Main results

Primary outcome
One trial using a modified maintenance of wakefulness test showed an improvement by 5.70 (95% confidence intervals 0.1 to 11.3) minutes more in the modafinil than the control group.

Secondary outcomes
In a double-blind crossover study of 10 participants with myotonic dystrophy, there was no difference between the selegiline and placebo periods in mean improvement in the multiple sleep latency test. Two trials, involving 60 participants in total, evaluated the efficacy and safety of modafinil in adults with myotonic dystrophy-related daytime sleepiness. The weighted mean difference on the Epworth Sleepiness Scale was -1.59 (95% confidence intervals, -2.77 to -0.42) in favour of modafinil.

Authors' conclusions

There is no evidence to support the routine use of psychostimulants to treat hypersomnia in myotonic dystrophy. There is some evidence from two studies that modafinil may improve daytime sleepiness. More randomised trials are needed to evaluate the efficacy and safety of psychostimulants.

top of page | contact us | about this site | disclaimer | privacy