EARLY MANAGEMENT OF DUCHENNE

MUSCULAR DYSTROPHY

A. Arampatzis,

I. Griparis,

S. Arampatzidou

The muscular dystrophies (MDs) are a

heterogeneous group of inherited
disorders characterized by progressive
weakness and degeneration
of skeletal muscles .

Duchenne MD

Duchenne muscular dystrophy is caused by the absence of dystrophin.

Dystrophin provides mechanical stability to the sarcolemma.Without

dystrophin, the sarcolemma becomes fragile and unable to withstand the
stress of normal muscle contractions. The resulting membrane damage
leads to fiber death or necrosis.

Necrotic fibers become replaced by fat and connective tissue, to such an

extent that there can be an apparent pseudohypertrophy, especially in
the calves.
(Lovering et al,2005)

The main aims of physiotherapy are to:

Maintain or improve muscle strength by

exercise.
Maximise functional ability by exercise and
the use of orthoses.
Minimise the development of contractures
by stretching and splinting.
(Eagle,2002)

Exercise in DMD

The role of strength training in MDs is

controversial, particularly with DMD.
Some of the widely held views are based
on evidence from studies with the mdx
mouse.
Care must be taken in extrapolating from
exercise studies in mdx mice to predicting
effects in DMD patients.
(Ansved, 2001)

Recommendations for exercise in DMD

Because of the increased susceptibility to

muscle damage, patients with MDshould be
advised to avoid exhaustive or maximal effort
during exercise(Petrof,1998).
Resistive eccentric exercises, which are
associated with muscle damage, should be
avoided( Friden et al, 2001).

Light to moderate resistive exercise can retard

the progression of muscle weakness without
negative effects.
Exercise combat the deconditioning that occurs
with immobility.(Sayers, 2000; Elder,1999).
Exercises may be more effective if introduced
when muscle weakness is not severe (Ansved,
2000; Hayes et al,1996).
The social and psychological aspects of
participation are very important. (Kroksmark,
1999).
Muscle training, should lead to an improved
functional ability for the patient in his daily life
activities.

Hydrotherapy

Swimming are often preferred rather than formal exercise programs.

For small children the goal is often just to have fun in the water.
Breast swimming is more difficult than swimming on the back.

Hydrotherapy is likely to result in minimal muscle damage, because

it minimizes the need for eccentric contractions.

Hydrotherapy appears especially useful in the later stages of DMD

in order to help maintain mobility in the absence of gravity.
(Kroksmark,1999;Kakulas,1999)

Contracture development

The primary problem in DMD is muscle

weakness.
Contractures develop secondary to this
weakness.

Orthoses

if healthy muscle is immobilised in a shortened position

sarcomeres will be lost,

if muscle is immobilised in a lengthened position

will add on sarcomeres.

Similarly in dystrophic mice a muscle immobilised in a

lengthened position is capable of adding on sarcomeres
but this is at a slower rate.

(Williams et al, 1990).

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Orthoses

A stretch of 30 min daily has been shown to prevent the

loss of sarcomeres and prevent much of the muscle
atrophy associated with immobilisation in the shortened
position
(Williams,1990)

The use of passive stretching and night splints were

more effective than passive stretching alone at both
delaying contracture development and prolonging
independent ambulation.

(Hyde,2000).
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Ambulant children

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Daily stretches to the gastrocnemiussoleus

complex, hip flexors and iliotibial band.
Encourage voluntary active exercise such as
swimming or hydrotherapy and cycling (may be
motor assisted).
Symmetry to be promoted in posture, exercises
and activities.
Eccentric activities such as running downhill
and excessive walking downstairs to be avoided.

Ambulant children

Night-time AFOs in addition to stretching daily

are recommended.

AFOs are not recommended for ambulant

children with DMD as this compromises their
ability to walk.

KAFOs can be used to prolong ambulation.

(Lovering et al,2005; Eagle,2003; Sharma et
al,1995 ).

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Non-ambulant children

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Mobilising passive or active assisted

exercises to maintain and promote
symmetry and comfort.
Symmetry to be promoted in posture
Daytime AFOs should be supplied
The benefit of a standing posture is logical
but not evidenced. Standing frames may
be used.

Life smiles to you!

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