European Journal of Neurology 2004, 11: 541–544 Prednisolone therapy in Duchenne muscular dystrophy prolongs ambulationand prevents scoliosis ¨ . Yılmaza, A. Karadumana and H. Topalog˘lub aHacettepe University School of Physiotherapy, Ankara, Turkey, and bDepartment of Child Neurology, Hacettepe Children’s Hospital, Steroids may have a beneficial effect on the course of Duchenne muscular dystrophy (DMD). However, results vary in different studies. This study consisted of 66 DMD boys who were in the therapy group and 22 DMD boys in the control group. The mean ages were 6.8 ± 2.1 years (range 2.5–12.5) and 7.0 ± 1.3 years (range 5.0–9.0), respectively. We assessed muscle strength, 10-m walking, ankle contracture, and loss of independent walking ability age and onset of scoliosis. Treatment regimen was oral prednisolone 0.75 mg/kg on alternate days, plus vitamin D 600–1200 units/day and acalcium-enriched diet. After a follow-up period of 2.75 ± 1.1 years (range 1.5–5) and when compared with controls, there was a statistically significant change in muscle strength between the two groups after 12 months (P < 0.05). Although 10-m walkingtime decreased in therapy group (P < 0.05), there was not significance between thegroups in the end. Boys in the control group developed significantly less ankle con-tractures (P < 0.05). None of the therapy group had scoliosis during the follow-upperiod (mean age 10.8 ± 1.2 years), whereas seven boys of the control group hadscoliosis at a mean age of 11.7 ± 2 years. Loss of walking ability age was statisticallydifferent between groups (P < 0.05). Our results indicate that, alternate-day pre-dnisolone regimen may prolong ambulation and scoliosis can be delayed or prevented.
necrosis through its immunosuppressive and anti- inflammatory effects (Kissel et al., 1991; Mesa et al., Duchenne muscular dystrophy (DMD) is one of the most 1992; Zatz et al., 1992). Although the benefits of severe and common neuromuscular diseases (Dubowitz, prednisone are evident in DMD patients, side-effects 1994; Matsumura and Campbell, 1994). Although major are a serious impairment for its widespread use (Feni- achievements have been accumulating recently regarding chel et al., 1991; Beckman and Henriksson, 1995).
the genetics and pathogenesis of DMD, no effective The aim of this study was to investigate the long-term medical treatment has been found yet. Physiotherapy, effects of the alternate-day prednisolone therapy results rehabilitation and use of steroids may prolong the on DMD particularly for muscle strength, contractures ambulatory period and therefore improve the quality of life (Drachman et al., 1974; Angelini and Bonifati, 2000).
Steroids have been demonstrated to be efficacious in slowing the progression of DMD and in delaying theloss of independent ambulation (Sansome et al., 1993; From the baseline, our study consisted of 66 DMD Bonifati et al., 2000; European Neuromuscular Centre, boys (mean age 6.8 ± 2.1 years; range 2.5–12.5) who 2000; Biggar et al., 2001; Fenichel et al., 2001). The were in the treatment group and 22 control DMD boys mechanisms by which such drugs might improve muscle (mean age 7.0 ± 1.3 years; range 5–9). Treatment strength and functional abilities are unknown. Corti- regimen was oral prednisolone 0.75 mg/kg on alternate costeroids may enhance myoblast proliferation and days, plus vitamin D 600–1200 units/day. A calcium- promote muscle regeneration. Alternatively, steroids enriched diet was given to both groups. Families gave their written consent for their children to participate lysosomal-bound proteases or muscle cell membranes.
into this study before the initiation. Age-matched con- Finally, prednisone could reduce muscle damage and trols were taken from an earlier cohort of patients, i.e.
pre-steroid era on a random basis.
The normal range of motion was assessed and goni- Correspondence: Prof. Haluk Topalog˘lu, Department of Child ometric measurements on the joints were performed, Neurology, Hacettepe Children’s Hospital, 06100 Ankara, Turkey(fax: +90 312 467 46 56; e-mail: htopalog@hacettepe.edu.tr).
Muscle strength was evaluated using Lovett’s manual Table 1 The demographic characteristics and comparisons of the muscle strength tests in seven muscle groups of the lower extremities (gluteus maximus, abductors and adductors of the hips, quadriceps femoris, hamstrings, tibialis anterior, gastrocnemius) and in eight muscle groups of the upper extremities (anterior and middle part of deltoid, middle and inferior part of trapezius, biceps brachii, triceps brachii, flexors and extensors of the wrist) (Daniels and Worthingham, 1972). Timed functional tests included 10-m walk; children’s 10-m walking time was noted to the second.
Instructions for families were first given when the patients were enrolled. This consisted of strengthening and stretching, posture and breathing exercises, posi- tioning and orthoses. In both groups, the children used bilateral polyethylene moulds (AFO) as a night Following their first evaluation, the patients were re- valued every 6 months and treatment programmes were modified according to the progress of the disease or improvement in function. Additionally, loss of ambulation ages and age of onset of scoliosis were recorded. Follow-up duration was 2.75 ± 0.1 years (range 1.5–5). In this study, statistical comparisons of functional and manual testing scores for both groups were given at the end of 12 months. However, for lossof ambulation and for scoliosis, the final follow-up date of each boy was taken into account and this was 1, Before treatment; 2, post-treatment 6th month; 3, post-treatment 12th month.
aFreidman test.
aOne-way ANOVA for sample size.
Results were expressed as mean ± SD. Student’s t-test The 10-m walking ability time was decreased statis- (ages, 10-m walking time, contractures of the ankles) tically significant in therapy group (P < 0.05); how- and Mann–Whitney U-tests (total scores of the upper ever, there was no significance between two groups in and lower extremities muscle strengths) were used for the end (P > 0.05) (Table 1; Fig. 1).
comparing two groups and sample-size ANOVA with Ankle contractures in the control group were repeated measures (10 m walking time, contractures of significantly less than the therapy group (P < 0.05).
the ankles) and Freidman tests (total scores of the up- A significant increase was found in therapy group per and lower extremities muscle strengths) were used ankle contractures during time (P < 0.05) (Table 1; for comparing within group. The statistical significance The ages of loss of walking were statistically signifi- cant between two groups (P < 0.05). Patients in thetherapy group lost walking ability at 10.0 ± 1.5 years (range 7–14) and at 8.6 ± 2.6 years (range 6–11) in the There was no statistically significant difference the control group. Fourteen boys were still walking in- mean ages of both groups (P < 0.05) (Table 1).
dependently after 12 years of age and three boys were There were no statistically significant difference in still walking after 13 years in the therapy group.
upper and lower extremitiesÕ total muscle strengths in However, all became off-feet by the end of age 14 years.
control group (P > 0.05); however, in the therapy We observed none of them had scoliosis above 24° during the follow-up period in the therapy group, (Table 1). Furthermore, there was a statistically signifi- whereas seven patients had scoliosis with a curve cant difference in total muscle strengths at the end of the above 45° at a mean age of 11.7 ± 0.8 years in the 12 months between two groups (P < 0.05) (Table 1).
Ó 2004 EFNS European Journal of Neurology 11, 541–544 Prednisolone therapy in Duchenne muscular dystrophy more in the steroid group compared with the control group; however, we found that this increase had no effect on the ambulation of the children.
Allsop and Ziter (1981) have stated that the per- formance of the activity is related to the strength of the involved muscles. Despite the increase in the time oftimed performance tests, important thing is the ability of children to preserve function or perform activities inthe test (Allsop and Ziter, 1981). In our study, the de- I: Before treatment
crease in the 10-m walking time was statistically signi- II: Post-treatment 6th month
ficant in the therapy group, and clinically our findings III: Post-treatment 12th month
We observed that the total muscle strengths of the upper and lower extremity were decreased at the end of the 12 months in the therapy group. These resultsshowed that, exercises were more effective than steroid Figure 1 Ten meter walking ability times changes in both groups.
treatment on improvement of the muscle strength.
Prolongation of the walking period in the therapygroup may denote that steroid treatment has a positive effect on the functions of the children.
The decrease in muscle strength and increase in 10-m walking time and ankle contractures in therapy group may be an indication that steroid treatment might have perceived by families as the most successful means on the treatment of DMD and by this reason exercises We have previously reported that long-term adminis- tration of prednisolone may have a protective effect on the spine (Tunca et al., 2001). This beneficial effect was I: Before treatment
II: Post-treatment 6th month
still present even after the child became unable to walk.
III: Post-treatment 12th month
Recently, similar findings have also been presented (King et al., 2003). We did not encounter any bone fractures or vertebral collapse. This is most likely be- cause of the addition of daily vitamin D on to our regi- Figure 2 Ankle contractures of the therapy and control groups.
men (European Neuromuscular Centre, 2000). At thisstage, we can only speculate that the beneficial effect ofsteroids on the spine may be due to the general well being of the child and to the increased quality of life measures.
In DMD, a combination of necrosis and progressive We would like to propose that the steroid adminis- insufficiency of muscle fibre regeneration is thought to tration on a continuum basis in DMD is effective for be responsible for replacement of muscle with connect- prolongation of ambulation period and delaying scoli- ive tissue and fat in later stages of disease (Zeman et al., 2000). During this period, important events take placeon the inflammatory cytokines. It has been shown that by depressing inflammation, the velocity of this pro-gressive event could be decreased (Blake and Kro¨ger, Allsop K, Ziter FA (1981). Loss of strength and functional 2000; Bonifati et al., 2000; European Neuromuscular decline in Duchenne’s dystrophy. Arch Neurol 38:406–411.
Angelini C, Bonifati DM (2000). New therapies in muscular Centre, 2000; Zeman et al., 2000; Matecki et al., 2001).
dystrophies. Neurol Sci 21:919–924.
In our cohort, independent walking period was pro- Beckman E, Henriksson KG (1995). Low-dose prednisolone longed and no scoliosis occured in the group that were treatment in Duchenne and Becker muscular dystrophy.
on steroids. Improvement in motor functions in DMD Neuromuscular Disorders 5:233–241.
patients maintains the quality of life and prevents other Biggar DW, Gingras M, Fehlings DL et al. (2001). Deflaza- cort treatment of Duchenne muscular dystrophy. The complications like scoliosis as it occurs at the late per- iod of the disease. Ankle contractures developed much Ó 2004 EFNS European Journal of Neurology 11, 541–544 Blake DJ, Kro¨ger S (2000). The neurobiology of Duchenne Matecki S, Topin N, Hayot M et al. (2001). A standardized muscular dystrophy: learning lessons from muscle. TINS method for evaluation of respiratory muscle endurance in patients with Duchenne muscular dystrophy. Neuromuscu- Bonifati M, Ruzza M, Bonometto P et al. (2000). A multicenter, double-blind, randomized tiral of deflazacort Matsumura K, Campbell KP (1994). Dystrophin–glycopro- versus prednisone in Duchenne muscular dystrophy. Muscle tein complex: its role in the molecular pathogenesis of muscular dystrophies. Muscle Nerve 17:2–15.
Daniels L, Worthingham C (1972). Muscle Testing. WB Mesa LE, Dubrowsky AL, Corderi J et al. (1992). Steroids in Duchenne muscular dystrophy. Neuromuscular Disorders Drachman DM, Toyka KV, Myer E (1974). Prednisone in Duchenne muscular dystrophy. Lancet 14:1409–1412.
Sansome A, Royston P, Dubowitz W (1993). Steroids in Dubowitz W (1994). The muscular dystrophies. In: Dubowitz Duchenne muscular dystrophy; pilot study of a new low- W ed. Muscle Disorders In Childhood. WB Saunders dosage schedule. Neuromuscular Disorders 3:567–569.
Tunca O, Kabakus N, Hergu¨ner O¨ et al. (2001). Alteranete Fenichel GM, Mendell JR, Moxley RT et al. (1991). A day prednisolone therapy in Duchenne muscular dystrophy.
comparison of daily and alternate-day prednisone therapy in the treatment of Duchenne muscular dystrophy. Arch European Neuromuscular Centre (2000). 2nd Workshop on the Treatment of Muscular Dystrophy, 10–12 December Fenichel GM, Griggs RC, Kissel J et al. (2001). A randomized 1999, Naarden, The Netherlands. Neuromuscular Disorders efficiacy and safety trial of oxandrolone in the tratment of Duchenne dystrophy. Neurology 56:1075–1079.
Zatz M, Pavanello RCM, Vainzof M et al. (1992). Steroids in King WM, Ruttencutter RE, Hoyle CJ et al. (2003). Ortho- Duchenne muscular dystrophy. Neuromuscular Disorders pedic effects of steroid threatment in Duchenne dystrophy.
Zeman RJ, Peng H, Danon MJ et al. (2000). Clenbuterol Kissel JT, Burrow KR, Rammohan KW et al. (1991).
reduces degeneration of exercised aged dystrophic (mdx) Mononuclear cell analysis of muscle biopsies in predni- sone-treated and untreated Duchenne muscular dystrophy.
Neurology 41:667–672.
Ó 2004 EFNS European Journal of Neurology 11, 541–544

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