Steroids prolong walking in Duchenne muscular dystrophy

A recent study, by Dr J Kissel and his colleagues at Ohio State University, has shown that the use of corticosteroids in Duchenne muscular dystrophy can prolong walking on average by three years as well as help prevent scoliosis. It appears that with these benefits, however, comes an increase in the number of bone fractures the boys are likely to suffer from.

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Steroids prolong walking in Duchenne muscular dystrophy

Corticosteroids have been in use for the management of Duchenne muscular dystrophy for a number of years now and are known to improve muscle strength and function as well as prolonging walking. There is, however, some concern about possible side effects from corticosteroid treatment that can make people cautious about their long-term use. Side effects can include weight gain, growth suppression, development of cataracts and thinning of the bones as well as several others. In order for parents to make an informed decision about whether or not their son should take corticosteroids it is important to have as much information as possible and the recently published study by Dr Kissel goes some way to providing a clearer idea of the potential benefits and risks of the long-term use of corticosteroids in Duchenne muscular dystrophy.

Dr Kissel carried out a study of 143 boys with Duchenne muscular dystrophy. 75 of the boys were on long-term steroid treatment and had been taking the corticosteroids for at least one year. 68 boys were in the “untreated” group and so had either not received any steroids or had taken them for less than 6 months. His main aim was to find out what effects, if any, long-term corticosteroid use would have on the bones of these boys.

The first benefit of corticosteroids described in the study was the confirmation of previous reports that corticosteroids could increase the time a boy with Duchenne is able to maintain independent walking. On average, the boys treated long-term with corticosteroids stayed walking for 3.3 years longer that the boys who did not receive steroid treatment.

The second benefit of the corticosteroids was in the prevention of scoliosis. Scoliosis is a curve of the spine so that instead of being in a straight line, the spine looks more like the letters ‘S’ or ‘C’. It occurs because the spinal muscles are not strong enough to support the spine and keep it in an upright position. Depending on the severity of the scoliosis, it can alter the shape of the chest and make breathing more difficult. It may also require surgery to correct it. Dr Kissel found that fewer boys in the corticosteroid-treated group had scoliosis than the untreated group. Of those boys who did have scoliosis, the curvature, or severity, was generally less in the boys treated with corticosteroids.

While the study showed a clear benefit from the corticosteroid treatment, it also demonstrated that the boys on long-term corticosteroid treatment were more likely to suffer from fractures in the long bones and in the vertebral bones than the untreated boys. The researchers were unsure as to why this difference occurred but suggested it might be because the boys taking long-term corticosteroids generally weighed more and remained walking for longer.

The current study by Dr Kissel provides more information and evidence about the precise effects of long-term corticosteroid use on boys with Duchenne and it is hoped this will help clinicians to advise parents so they can make a more informed decision about whether their son should be taking corticosteroids.




Further information and links

About Duchenne muscular dystrophy
For more information about this condition, go to our information pages

Steroids in Duchenne muscular dystrophy
For further information about corticosteroid use in Duchenne muscular dystrophy please go to our questions and answers page

Neurology
The paper referred to in the above article was published in the journal Neurology. Neurology is subscription only so the original article is not freely available. The article itself is written in medically technical language with no summary in layman's terms.