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Neuromuscular Disorders : NMD Dec 2010The complexity and variability of disease manifestations in myotonic dystrophy (DM1) pose a challenge for the clinical management of patients. The follow-up of DM1...
The complexity and variability of disease manifestations in myotonic dystrophy (DM1) pose a challenge for the clinical management of patients. The follow-up of DM1 patients has been described as fragmented, inadequate or even deficient for many patients. Through a systematic review of the medical and social literature and a validation process with a DM1 expert panel, we summarized systemic and social concerns clinically relevant to DM1 and revisited recommendations for treatment. This article summarizes common manifestations of the central nervous system, visual, respiratory, cardiac, gastro-intestinal, genito-urinary, muscular and metabolic impairments. In addition, we emphasized the social features of DM1 such as low education attainment, low employment, poor familial and social environment and poor social participation. While cardiac, respiratory and swallowing problems affect life expectancy, it is often excessive daytime sleepiness, fatigue, gastro-intestinal and cognitive behavioural manifestations that are the most disabling features of the disorder. A more holistic approach in the management of DM1 and a purposeful integrated organization of care involving all members of the patients' environment including family, clinicians, decision-makers and community organizations are needed to move out of the spiral of disease and handicap and move toward optimal citizenship and quality of life.
Topics: Adult; Humans; Myotonic Dystrophy; Severity of Illness Index; Social Environment; Social Participation
PubMed: 20884209
DOI: 10.1016/j.nmd.2010.08.006 -
The Cochrane Database of Systematic... Jan 2010Strength training or aerobic exercise programmes might optimise muscle and cardiorespiratory function and prevent additional disuse atrophy and deconditioning in people... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Strength training or aerobic exercise programmes might optimise muscle and cardiorespiratory function and prevent additional disuse atrophy and deconditioning in people with a muscle disease.
OBJECTIVES
To examine the safety and efficacy of strength training and aerobic exercise training in people with a muscle disease.
SEARCH STRATEGY
We searched the Cochrane Neuromuscular Disease Group Trials Specialized Register (July 2009), the Cochrane Rehabilitation and Related Therapies Field Register (October 2002, August 2008 and July 2009), The Cochrane Central Register of Controlled Trials (The Cochrane Library Issue 3, 2009) MEDLINE (January 1966 to July 2009), EMBASE (January 1974 to July 2009), EMBASE Classic (1947 to 1973) and CINAHL (January 1982 to July 2009).
SELECTION CRITERIA
Randomised or quasi-randomised controlled trials comparing strength training or aerobic exercise programmes, or both, to no training, and lasting at least 10 weeks.For strength training Primary outcome: static or dynamic muscle strength. Secondary: muscle endurance or muscle fatigue, functional assessments, quality of life, muscle membrane permeability, pain and experienced fatigue.For aerobic exercise training Primary outcome: aerobic capacity expressed as work capacity. Secondary: aerobic capacity (oxygen consumption, parameters of cardiac or respiratory function), functional assessments, quality of life, muscle membrane permeability, pain and experienced fatigue.
DATA COLLECTION AND ANALYSIS
Two authors independently assessed trial quality and extracted the data.
MAIN RESULTS
We included three trials (121 participants). The first compared the effect of strength training versus no training in 36 people with myotonic dystrophy. The second trial compared strength training versus no training, both combined with albuterol or placebo, in 65 people with facioscapulohumeral muscular dystrophy. The third trial compared combined strength training and aerobic exercise versus no training in 18 people with mitochondrial myopathy. In the myotonic dystrophy trial there were no significant differences between training and non-training groups for primary and secondary outcome measures. In the facioscapulohumeral muscular dystrophy trial only a +1.17 kg difference (95% confidence interval 0.18 to 2.16) in dynamic strength of elbow flexors in favour of the training group reached statistical significance. In the mitochondrial myopathy trial there were no significant differences in dynamic strength measures between training and non-training groups. Exercise duration and distance cycled in a submaximal endurance test increased significantly in the training group compared to the control group.
AUTHORS' CONCLUSIONS
In myotonic dystrophy and facioscapulohumeral muscular dystrophy, moderate-intensity strength training appears not to do harm but there is insufficient evidence to conclude that it offers benefit. In mitochondrial myopathy, aerobic exercise combined with strength training appears to be safe and may be effective in increasing submaximal endurance capacity. Limitations in the design of studies in other muscle diseases prevent more general conclusions in these disorders.
Topics: Exercise; Humans; Mitochondrial Myopathies; Muscular Dystrophy, Facioscapulohumeral; Myotonic Dystrophy; Physical Fitness; Randomized Controlled Trials as Topic
PubMed: 20091552
DOI: 10.1002/14651858.CD003907.pub3 -
Disability and Rehabilitation 2009A systematic evaluation of the literature to identify health and contextual factors associated with employment in patients with neuromuscular diseases (NMD) and to... (Review)
Review
Classification of employment factors according to the International Classification of Functioning, Disability and Health in patients with neuromuscular diseases: a systematic review.
PURPOSE
A systematic evaluation of the literature to identify health and contextual factors associated with employment in patients with neuromuscular diseases (NMD) and to perform a best evidence synthesis, taking into account the design of studies, methodological quality and the statistical significance of findings.
METHOD
Publications were retrieved by a computerised search in medical and psychological databases. Two reviewers assessed titles and abstracts first and assessed the quality of the remaining full text publications independently as well. Of the residual publications, health and contextual factors associated with employment in patients with NMD were extracted. The factors found were included in a recently developed expanded International Classification of Functioning, Disability and Health scheme.
RESULTS
Six hundred and sixty-two titles and abstracts were screened. The main reason to exclude a title and/or abstract was the absence of the study population selected: Facioscapulohumeral Muscular Dystrophy (FSHD), Hereditary Motor and Sensory Neuropathy (HMSN) & Myotonic Dystrophy (MD). Of the remaining 20 full-text publications, eight publications fulfilled the inclusion criteria: two repeated survey designs and six cross-sectional studies. Factor extraction resulted in 94 factors related to employment. Ten factors in five publications were indicative for an association with employment status: Disease related factors HMSN, MD & NMD in general), factors related to functions (physical functions, muscle power functions), general personal factors (age, gender and education), work related personal factors (type of occupation, and expressed interest in employment by patients with NMD).
CONCLUSION
In the best evidence synthesis ten factors were indicative for an association with employment status in patients with NMD in five publications with good to excellent methodological quality.
Topics: Disabled Persons; Employment; Hereditary Sensory and Motor Neuropathy; Humans; Muscular Dystrophy, Facioscapulohumeral; Myotonic Dystrophy; Neuromuscular Diseases; Vocabulary, Controlled
PubMed: 19903125
DOI: 10.3109/09638280902951838 -
The Cochrane Database of Systematic... Jul 2009"Foot drop" or "Floppy foot drop" is the term commonly used to describe weakness or contracture of the muscles around the ankle joint. It may arise from many... (Review)
Review
BACKGROUND
"Foot drop" or "Floppy foot drop" is the term commonly used to describe weakness or contracture of the muscles around the ankle joint. It may arise from many neuromuscular diseases.
OBJECTIVES
To conduct a systematic review of randomised trials for the treatment of foot drop resulting from neuromuscular disease.
SEARCH STRATEGY
In this update, we searched the Cochrane Neuromuscular Disease Group Trials Register (April 2009), MEDLINE (January 1966 to April 24 2009), EMBASE January 1980 to April 24 2009), CINAHL (January 1982 to May 6 2009), AMED (January 1985 to April 24 2009), the British Nursing Index (January 1985 to January 2008) and Royal College of Nursing Journal of Databases (January 1985 to January 2008).
SELECTION CRITERIA
Randomised and quasi-randomised trials of physical, orthotic and surgical treatments for foot drop resulting from lower motor neuron or muscle disease and related contractures were included. People with primary joint disease were excluded. Interventions included a 'wait and see' approach, physiotherapy, orthoses, surgery and pharmacological therapy. The primary outcome measure was quantified ability to walk whilst secondary outcome measures included range of movement, dorsiflexor torque and strength, measures of activity and participation, quality of life and adverse effects.
DATA COLLECTION AND ANALYSIS
Methodological quality was evaluated by two authors using the van Tulder criteria. Four studies with a total of n = 152 participants were included in the update to the original review. Heterogeneity of the studies precluded pooling the data.
MAIN RESULTS
Early surgery did not significantly affect walking speed in a trial including 20 children with Duchenne muscular dystrophy. Both groups deteriorated during the 12 months follow-up. After one year, the mean difference (MD) of the 28 feet walking time was 0.00 seconds (95% confidence interval (CI) -0.83 to 0.83) and the MD of the 150 feet walking time was -2.88 seconds, favouring the control group (95% CI -8.18 to 2.42). Night splinting of the ankle did not significantly affect muscle force or range of movement about the ankle in a trial of 26 participants with Charcot-Marie-Tooth disease. Improvements were observed in both the splinting and control groups. In a trial of 26 participants with Charcot-Marie-Tooth disease and 28 participants with myotonic dystrophy, 24 weeks of strength training significantly improved six-metre timed walk in the Charcot-Marie-Tooth group compared to the control group (MD 0.70 seconds, favouring strength training, 95% CI 0.23 to 1.17), but not in the myotonic dystrophy group (MD -0.20 seconds, favouring the control group, 95% CI -0.79 to 0.39). No significant differences were observed for the 50 metre timed walk in the Charcot-Marie-Tooth disease group (MD 1.90 seconds, favouring the training group, 95% CI -0.29 to 4.09) or the myotonic dystrophy group (MD -0.80 seconds, favouring the control group, 95% CI -5.29 to 3.69). In a trial of 65 participants with facioscapulohumeral muscular dystrophy, 26 weeks of strength training did not significantly affect ankle strength. After one year, the mean difference in maximum voluntary isometric contraction was -0.43 kg, favouring the control group (95%CI -2.49 to 1.63) and the mean difference in dynamic strength was 0.44 kg, favouring the training group (95%CI -0.89 to 1.77).
AUTHORS' CONCLUSIONS
Only one study, involving people with Charcot-Marie-Tooth disease, demonstrated a statistically significant positive effect of strength training. No effect of strength training was found in people with either myotonic dystrophy or facioscapulohumeral muscular dystrophy. Surgery had no significant effect in children with Duchenne muscular dystrophy and night splinting of the ankle had no significant effect in people with Charcot-Marie-Tooth disease. More evidence generated by methodologically sound trials is required.
Topics: Charcot-Marie-Tooth Disease; Child; Exercise Therapy; Gait Disorders, Neurologic; Humans; Male; Muscle Weakness; Muscular Dystrophy, Duchenne; Myotonic Dystrophy; Resistance Training; Treatment Outcome; Walking
PubMed: 19588347
DOI: 10.1002/14651858.CD003908.pub3 -
Archives of Physical Medicine and... Nov 2007To summarize and critically appraise the available evidence on exercise therapy and other types of physical therapies for patients with neuromuscular diseases (NMD). (Review)
Review
OBJECTIVE
To summarize and critically appraise the available evidence on exercise therapy and other types of physical therapies for patients with neuromuscular diseases (NMD).
DATA SOURCES
Cochrane Central Register of Controlled Trials and Cochrane Database of Systematic Reviews, Medline, CINAHL, EMBASE (Rehabilitation and Physical Medicine), and reference lists of reviews and articles.
STUDY SELECTION
Randomized clinical trials (RCTs), controlled clinical trials (CCTs), and other designs were included. Study participants had to have any of the following types of NMD: motoneuron diseases, disorders of the motor nerve roots or peripheral nerves, neuromuscular transmission disorders, or muscle diseases. All types of exercise therapy and other physical therapy modalities were included. Outcome measures had to be at the level of body functions, activities, or participation according to the definitions of the International Classification of Functioning, Disability and Health (ICF).
DATA EXTRACTION
Two reviewers independently decided on inclusion or exclusion of articles and rated the methodologic quality of the studies included. All RCTs, CCTs, and other designs only if of sufficient methodologic quality were included in a best evidence synthesis. A level of evidence was attributed for each subgroup of NMD and each type of intervention.
DATA SYNTHESIS
Initially 58 studies were included: 12 RCTs, 5 CCTs, and 41 other designs. After methodologic assessment, 19 other designs were excluded from further analysis. There is level II evidence ("likely to be effective") for strengthening exercises in combination with aerobic exercises for patients with muscle disorders. Level III evidence ("indications of effectiveness") was found for aerobic exercises in patients with muscle disorders and for the combination of muscle strengthening and aerobic exercises in a heterogeneous group of muscle disorders. Finally, there is level III evidence for breathing exercises for patients with myasthenia gravis and for patients with myotonic muscular dystrophy. Adverse effects of exercise therapy were negligible.
CONCLUSIONS
The available evidence is limited, but relevant for clinicians. Future studies should be preferably multicentered, and use an international classification of the variables of exercise therapy and an ICF core set for NMD in order to improve comparability of results.
Topics: Controlled Clinical Trials as Topic; Evidence-Based Medicine; Exercise Therapy; Humans; Neuromuscular Diseases; Outcome and Process Assessment, Health Care; Physical Therapy Modalities; Randomized Controlled Trials as Topic
PubMed: 17964887
DOI: 10.1016/j.apmr.2007.07.024 -
The Cochrane Database of Systematic... Apr 2007"Foot drop" or "Floppy foot drop" is the term commonly used to describe weakness or contracture of the muscles around the ankle joint. It may arise from many... (Review)
Review
BACKGROUND
"Foot drop" or "Floppy foot drop" is the term commonly used to describe weakness or contracture of the muscles around the ankle joint. It may arise from many neuromuscular diseases.
OBJECTIVES
To conduct a systematic review of randomised trials of treatment for footdrop resulting from neuromuscular disease.
SEARCH STRATEGY
We searched the Cochrane Neuromuscular Disease Group Trials Register (July 2005), MEDLINE (January 1966 to July 2005), EMBASE (January 1980 to July 2005), AMED (January 1985 to July 2005) and CINAHL databases (January 1982 to July 2005).
SELECTION CRITERIA
Randomised and quasi-randomised trials of physical, orthotic and surgical treatments for footdrop resulting from lower motor neuron or muscle disease and related contractures were included. People with primary joint disease were excluded. Interventions included a 'wait and see' approach, physiotherapy, orthotics, surgery and pharmacological therapy. The primary outcome measure was ability to walk whilst secondary outcome measures included dorsiflexor torque and strength, measures of 'activity' and 'participation' and adverse effects.
DATA COLLECTION AND ANALYSIS
Methodological quality was evaluated by two authors using the van Tulder criteria. Three studies with altogether 139 participants were included in the review. Heterogeneity of the studies precluded pooling the data.
MAIN RESULTS
Early surgery did not significantly affect walking speed in a trial including 20 children with Duchenne muscular dystrophy. After one year, the mean difference (MD) of the 28 feet walking time was 0.00 seconds (95% confidence interval (CI) -0.83 to 0.83) and the MD of the 150 feet walking time was -2.88 seconds, (95% CI -8.18 to 2.42). In a trial with altogether 26 participants with Charcot-Marie-Tooth disease (hereditary motor and sensory neuropathy), long-term strength training significantly increased walking speed on a 6 metre timed walk (MD -0.70 seconds, 95% CI -1.17 to -0.23) but not on a 50 metre timed walk (MD -1.9 seconds, 95% CI -4.09 to 0.29). In a trial of a 24-week strength training programme in 28 participants with myotonic dystrophy, there was no significant change in walking speed on either a 6 or 50 metre walk.
AUTHORS' CONCLUSIONS
Using the primary outcome of ability to walk, only one study demonstrated a positive effect and that was an exercise programme for people with Charcot-Marie-Tooth disease. Surgery was not significantly effective in children with Duchenne Muscular Dystrophy. More evidence generated by methodologically sound trials is required.
Topics: Charcot-Marie-Tooth Disease; Child; Exercise Therapy; Gait Disorders, Neurologic; Humans; Male; Muscular Dystrophy, Duchenne; Treatment Outcome; Walking
PubMed: 17443532
DOI: 10.1002/14651858.CD003908.pub2 -
The Cochrane Database of Systematic... Jul 2006Excessive daytime sleepiness is a common symptom of myotonic dystrophy. Psychostimulants are drugs increasingly used to treat hypersomnia in myotonic dystrophy. (Meta-Analysis)
Meta-Analysis Review
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.
Topics: Benzhydryl Compounds; Disorders of Excessive Somnolence; Humans; Modafinil; Monoamine Oxidase Inhibitors; Myotonic Dystrophy; Psychotropic Drugs; Randomized Controlled Trials as Topic; Selegiline
PubMed: 16855999
DOI: 10.1002/14651858.CD003218.pub2 -
The Cochrane Database of Systematic... Jan 2006Abnormal delayed relaxation of skeletal muscles, known as myotonia, can cause disability in myotonic disorders. Sodium channel blockers, tricyclic antidepressive drugs,... (Review)
Review
BACKGROUND
Abnormal delayed relaxation of skeletal muscles, known as myotonia, can cause disability in myotonic disorders. Sodium channel blockers, tricyclic antidepressive drugs, benzodiazepines, calcium-antagonists, taurine and prednisone may be of use in reducing myotonia.
OBJECTIVES
To consider the evidence from randomised controlled trials on the efficacy and tolerability of drug treatment in patients with clinical myotonia due to a myotonic disorder.
SEARCH STRATEGY
We searched the Cochrane Neuromuscular Disease Group trials register (April 2004), MEDLINE (January 1966 to December 2003) and EMBASE (January 1980 to December 2003). Grey literature was handsearched and reference lists of identified studies and reviews were examined. Authors, disease experts and manufacturers of anti-myotonic drugs were contacted.
SELECTION CRITERIA
We considered all (quasi) randomised trials of participants with myotonia treated with any drug treatment versus no therapy, placebo or any other active drug treatment. The primary outcome measure was:reduced clinical myotonia using two categories: (1) no residual myotonia or improvement of myotonia or (2) No change or worsening of myotonia. Secondary outcome measures were:(1) clinical relaxation time; (2) electromyographic relaxation time; (3) stair test; (4) presence of percussion myotonia; and (5) proportion of adverse events.
DATA COLLECTION AND ANALYSIS
Two authors extracted the data independently onto standardised extraction forms and disagreements were resolved by discussion.
MAIN RESULTS
Nine randomised controlled trials were found comparing active drug treatment versus placebo or another active drug treatment in patients with myotonia due to a myotonic disorder. Included trials were double-blind or single-blind crossover studies involving a total of 137 patients of which 109 had myotonic dystrophy type 1 and 28 had myotonia congenita. The studies were of poor quality. Therefore, we were not able to analyse the results of all identified studies. Two small crossover studies without a washout period demonstrated a significant effect of imipramine and taurine in myotonic dystrophy. One small crossover study with a washout period demonstrated a significant effect of clomipramine in myotonic dystrophy. Meta-analysis was not possible.
AUTHORS' CONCLUSIONS
Due to insufficient good quality data and lack of randomised studies, it is impossible to determine whether drug treatment is safe and effective in the treatment of myotonia. Small single studies give an indication that clomipramine and imipramine have a short-term beneficial effect and that taurine has a long-term beneficial effect on myotonia. Larger, well-designed randomised controlled trials are needed to assess the efficacy and tolerability of drug treatment for myotonia.
Topics: Humans; Myotonia; Myotonic Dystrophy; Randomized Controlled Trials as Topic
PubMed: 16437496
DOI: 10.1002/14651858.CD004762.pub2 -
The Cochrane Database of Systematic... Jan 2005Strength training or aerobic exercise programmes might maximise muscle and cardiorespiratory function and prevent additional disuse atrophy in patients with muscle... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Strength training or aerobic exercise programmes might maximise muscle and cardiorespiratory function and prevent additional disuse atrophy in patients with muscle disease. However, over-exerting might cause more rapid disease progression.
OBJECTIVES
To examine the efficacy and safety of strength training and aerobic exercise training in patients with muscle diseases.
SEARCH STRATEGY
We searched the Cochrane Neuromuscular Disease Group register (October 2002 and May 2004), the Cochrane Collaboration Rehabilitation and Related Therapies Field register (October 2002), MEDLINE (January 1966 to December 2002), EMBASE (January 1973 to October 2002), and CINAHL (January 1982 to August 2002) for randomised trials. We reviewed the bibliographies of trials identified and reviews covering the subject.
SELECTION CRITERIA
Randomised or quasi-randomised controlled trials comparing strength training and/or aerobic exercise programmes lasting at least 10 weeks. Types of outcome measures: FOR STRENGTH TRAINING. Primary: static or dynamic muscle strength. Secondary: muscle strength (endurance or fatigue), functional assessments, quality of life, muscle membrane permeability, pain, and fatigue. FOR AEROBIC EXERCISE TRAINING. Primary: aerobic capacity expressed as work capacity. Secondary: aerobic capacity (oxygen consumption, parameters of cardiac or respiratory function), functional assessments, quality of life, muscle membrane permeability, pain, and fatigue.
DATA COLLECTION AND ANALYSIS
Two reviewers independently assessed trial quality and extracted the data.
MAIN RESULTS
We identified two randomised trials fulfilling all inclusion criteria. The first trial compared the effect of strength training versus no training in 36 patients with myotonic dystrophy. The other trial compared strength training versus no training combined with albuterol or placebo in 65 patients with facioscapulohumeral muscular dystrophy. Methodological quality and training programmes were graded adequate. In the myotonic dystrophy trial there were no significant differences between training and non-training groups for the primary outcome measure. In the facioscapulohumeral muscular dystrophy trial static muscle strength did not show significant differences between training and non-training groups. Only a +1.2 kg difference (95% confidence interval 0.2 to 2.1) in dynamic strength of elbow flexors in favour of the training group, reached statistical significance. For both trials there were no significant differences between groups for most of the secondary outcome measures, including those covering adverse effects.
AUTHORS' CONCLUSIONS
In myotonic dystrophy and facioscapulohumeral muscular dystrophy moderate-intensity strength training appears not to do harm but there is insufficient evidence to establish that it offers benefit. Limitations in the design of studies in other muscle diseases prevent general conclusions in these disorders.
Topics: Exercise; Humans; Muscular Dystrophies; Myotonic Dystrophy; Physical Fitness; Randomized Controlled Trials as Topic
PubMed: 15674918
DOI: 10.1002/14651858.CD003907.pub2 -
The Cochrane Database of Systematic... 2002Excessive daytime sleepiness is a common symptom of myotonic dystrophy. Psychostimulants are drugs increasingly used to treat hypersomnia in myotonic dystrophy. (Review)
Review
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 Trial Register (searched May 2001) for randomised trials concerning psychostimulants in myotonic dystrophy, we searched of the bibliographies of identified papers and we contacted the authors of the papers.
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 reviewers and the selection of eligible studies was agreed by them and a third reviewer. Data were extracted by one reviewer and checked by a second reviewer.
MAIN RESULTS
Primary outcome We found no trial that assessed the effect of a psychostimulant on the results of the maintenance of wakefulness tests. Secondary outcomes Only one eligible trial was found. In this crossover double blind study of 10 patients with myotonic dystrophy, the efficacy of selegiline was evaluated against a placebo on the multiple sleep latency test. There was no difference between the selegiline and placebo periods in mean improvement in the multiple sleep latency test scale.
REVIEWER'S CONCLUSIONS
There is no evidence to support the use of a psychostimulant to treat hypersomnia in myotonic dystrophy. Randomised trials are needed to evaluate the efficacy and safety of psychostimulants.
Topics: Disorders of Excessive Somnolence; Humans; Monoamine Oxidase Inhibitors; Myotonic Dystrophy; Psychotropic Drugs; Randomized Controlled Trials as Topic; Selegiline
PubMed: 12519589
DOI: 10.1002/14651858.CD003218