Anna MoriartySteps Neurological Physiotherapy

Explain the role of physiotherapy in the management of muscular dystrophy

Outline common problems

Provide guidelines for management

Discuss outcomes of relevant research trials

Monitor over time: Joint range Muscle length Posture Functional activities

Prescribe (and monitor): Exercises Equipment

Communicate with health team

◦ Muscle weakness

◦ Muscle shortening

◦ Loss of function

◦ Respiratory complications

◦ Children – interference with motor development

Individuals with neuromuscular diseases are less active than the general population (often termed ‘sedentary lifestyle’)

The disease process causes a reduction in functional muscle mass and strength

Disuse atrophy – preferential to fibre type Decreased strength Reduced cardiopulmonary function Reduced resting energy expenditure Greater age related decline in muscle mass

and cardiopulmonary function Predisposes to obesity; OA; T2DM; HT; OP;

Heart Disease .....

Disease affected atrophic fibres are scattered throughout the muscle

A weak muscle must contract at a higher percentage of its max strength to perform the same activity –therefore will fatigue in a shorter time

The relative amount of disease affected fibres will determine the degree of weakness, and also the potential for trainability

Strength training is likely to reverse effects of disuse atrophy, but there is no evidence it brings about change in disease affected muscle fibres

Imbalance in muscle strength – difficult to exercise strong muscles without risking overuse of weak muscles also involved in the movement

Daily activities may be working your muscles at their maximal level already

Are not recommended in fast progressive MD

Place significant stress on the muscle cell membrane – this may cause damage to the muscle fibre and hasten muscle weakness

May cause the muscle’s pool of satellite cells to be used up prematurely

Kilmer 2001 Study – single bout of eccentric exercise

Clinical trials are important

They must be well designed to give useful information

Powerful clinical trials are difficult to conduct in the FSHD and LGMD populations

Aerobic training:◦ Olsen et al 2005 (FSHD) Positive results for 12 weeks cycle training ◦ Sveen et al, 2007 (LGMD) Positive results for 12 weeks cycle training◦ Kilmer, 2002 - review Cardiorespiratory adaptions to submaximal aerobic

exercise training are similar to in able bodied persons There is significant variation between individuals Long term effect is not known

Strength training:◦ Vignos & Watkins, 1966 Max resistance strength training for 1 year Significant improvement with plateau at 4 months Stronger muscles improved more

◦ van der Kooi, 2004 – RCT 1 year strength training at a moderate level for 65 persons with

FSHD Small improvevents in strength, no signs of overuse weakness

◦ Sveen et al 2008◦ 11 Becker MD, cycled 50 x 30 min over 12 weeks @ 65% Vo2◦ Increased exercise performance and daily function

◦ Blain et al, 2010◦ Eccentric contraction does damage muscle fibres in MD mice

◦ DMD – ‘no use is disuse’ study (low intensity exercise) –results expected 2011

There is currently no evidence that physical training of any sort can influence the evolution of muscular dystrophies in the long term.

However, small improvements have been demonstrated with aerobic and resistance training in slowly progressive MD

Stronger muscles gain more strength with training

Overuse weakness appears no not play a significant role in slowly progressive MD

Muscles monitored:

◦ Hip flexors◦ Hip abductors◦ Hamstrings◦ Calves◦ Elbow flexors◦ Wrist flexors

These muscle groups are commonly tight in the MD population

Stretches Night splinting (Hyde et al, 2000) Standing frames Serial casting Appropriate seating (prevent length

imbalance in spinal muscles)

Must be individually tailored

Must be regularly monitored / adjusted

Should involve activities that interest the individual

Should take into account the activity already performed each day (ie school activity; walking to the train station each morning)

Aim for a moderate level of aerobic activity

Target a low-moderate level of resistance for strength training

Muscles should have antigravity movement to be ‘eligible’ for strength training – this also applies to the opposing muscle group(minimum grade 3 strength)

Rapidly progressive MD – the jury is out

Aerobic◦ Bike◦ Walk◦ Swim◦ Tai chi

Strength◦ Machine weights◦ Dumbbells / cuff weights◦ Theraband◦ Gravity

Stretch◦ Muscle groups exercised◦ Muscle groups prone to contractions

Practice functional activities