Rehabilitation in Myopathies PMR Refresher Course

1. Rehabilitation in myopathies
Venugopal Kochiyil
Medical Head of the Unit - Northern Adelaide
Rehabilitation Service
Modbury Hospital
South Australia, Australia

2. Myopathies
• A group of disorders affecting skeletal muscle
producing weakness, fatigue and deformities
• Can be associated with involvement of other systems
• Can be dystrophic, congenital, metabolic,
inflammatory, endocrine, toxic or steroid induced

3. Clinical pearls in diagnosis
• Pattern of weakness, wasting, hypertrophy
• Course of weakness – acute, chronic, episodic
• Progression of weakness
• Onset
• Muscle cramps, stiffness
• Sensations
• Gait abnormalities
• Functional difficulties
• History of recent illness
• Feeding difficulties
• Cardiac symptoms, pulmonary symptoms
• Developmental history
• Family history (AD, AR, X-linked)
Myopathic Disorders in Physical Med and Rehabilitation (Braddom RL, 2011)

4. Rehabilitation in myopathies
• Identify impairments, activity limitations and
participation restrictions
• Goal orientated (maximise functions, maintain
mobility, prevent physical deformities, prevent
medical complications, social life)
• A multidisciplinary/interdisciplinary approach

5. Physical training
• Eccentric contraction v/s concentric contractions
• Muscle groups doing eccentric activity are affected
first in many myopathies
• Resistance exercises are not harmful
• A submaximal resistance exercise program could be
tried
• Assisted exercise training
• Supervised or not supervised

6. Exercises
• Voluntary active exercises like swimming
• Limited by perceived exertion
• Mechanism - preventing disuse, enhance myofiber
repair, decreasing muscle fibrosis and production of
antioxidants
• Fatigue is equally important and need to be
differentiated from muscle weakness
• Aerobic training

7. Evidence
• Moderate-intensity strength training in myotonic
dystrophy and FSHD and aerobic exercise training in
dermatomyositis and polymyositis and myotonic
dystrophy type I appear to do no harm, but there is
insufficient evidence to conclude that they offer 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
9 Jul 2013 | DOI: 10.1002/14651858.CD003907.pub4

8. Contractures
• Contractures – myogenic, arthrogenic or soft tissue
• High risk in dystrophinopathies
• To prevent contractures – regular standing and
walking, passive stretching as a home program,
positioning to promote extension and night splinting
• Use of wheelchair accelerate contractures

9. Polymyositis/Dermatomyositis
• Idiopathic inflammatory myositis
• Female to male 2:1
• Evidenced by proximal muscle weakness (subacute) and
inflammation
• Distal muscle groups are also involved
• DM has characteristic skin findings which occurs prior or
along with weakness (in 60%), muscle tenderness in up to
50%.
• Associated with Interstitial pulmonary disease, dysphagia,
polyarthritis, myocarditis, risk of malignancy
• Overlap syndromes

10. Diagnosis
• Elevated muscle enzymes (CK, LDH, ALT, AST)
• Correlation between CK and muscle involvement.
• May be normal in DM
• Elevated CK MB in the absence of myocarditis (Do troponins in
this case)
• ANA
• Myositis specific antibodies (30%) – anti Jo 1, anti SRP, anti M2
• EMG
• MRI
• Biopsy

11. Prognosis
• Delay in the initiation of treatment for more than six
months after symptom onset
• Greater weakness at presentation
• The presence of dysphagia
• Respiratory muscle weakness
• Interstitial lung disease
• Associated malignancy
• Cardiac involvement
• ? Increased CK
www.uptodate.com

12. Management
• Glucocorticoids – start with oral or pulse IV
• Once the disease is under control, taper to lowest
effective dose for atleast one year
• No standard tapering regimen
• Glucocorticoid sparing agents – Azothiaprine, MTX
• IVIg – def role in resistant and recurrent presentation

13. Issues to consider
• Steroid induced myopathy
• Steroid induced osteoporosis
• Infections
• Aspiration

14. Therapy
• Therapy according to the severity of disease process
• Passive range of motion exercises
• Positioning to prevent contractures and pressure sores
• Easy to moderate resistive exercises in acute group
• Moderate to intensive resistive and aerobic exercises
in chronic group
Alexanderson H, Lundberg IE. Curr Opin Rheumatol 2012;24 www.co-rheumatology.org

15. Inclusion Body myositis
• Rare sporadic disorder
• Affect elderly men
• Insidious onset of weakness/ history of falls
• Proximal and distal muscle weakness (asymmetric
weakness)
• Facial muscle involvement
• Occasional myalgia
• Muscle atrophy
• Dysphagia could be a presenting complaint

16. Diagnosis
• History, examination
• Ask for history of drugs and other substances
• Family history of hereditary myopathies
• Increased muscle enzymes
• Muscle biopsy – rimmed vacuoles, mononuclear
inflammatory cells invading non necrotic muscle

17. Management
• Falls prevention, assistive devices, AFOs
• Immunomodulatory therapy ?
• IVIgs
• Swallow assessment
• Nutritional support
• Low resistance, endurance exercises
• Passive ROM exercises

18. Prognosis
• Tend to progress over time
• Faster progression in elderly patients
• Significant disability within 15 years of diagnosis

19. Statin induced myopathy
• Approx 0.1% of population
• Presents with myalgia and weakness
• Within weeks and months after statin initiation
• Possibly related to reduction in the synthesis of Co Q10
• Type of statin is important
• Avoid statin in pre existing neuromuscular weakness
• Higher risk in Hypothyroidism, renal failure and
obstructive liver disease
• CK level

20. Prognosis
• Start recovering once statin is stopped
• Usually recover within 6 months

21. Critical illness myopathy
• Critical illness neuropathy, myopathy or both
• Muscle weakness, failure to wean, prolonged
ventilation
• 25-83%
• Proximal weakness and wasting in myopathy
• Higher in trauma, sepsis, steroid use, neuromuscular
blockade drug use in ICU

22. Criteria for myopathy (Latronico 2011)
• Individual is critically ill (multi-organ involvement)
• Limb weakness and or difficulty in weaning off
ventilator
• CMAP less than 80% without conduction block in
atleast two nerves
• Sensory action potential more than 80%
• Myopathic pattern in needle EMG
• Absence of decremental pattern in repetitive
stimulation
• Muscle biopsy shows primary muscle pathology

23. Criteria for CIP
• 1 and 2 criteria are the same
• Electrophysiological evidence of axonal motor and
sensory neuropathy
• Absence of decremental response

24. Prognosis
• 50% near complete recovery
• Residual impairments in severely affected

25. Physical rehab
• Early mobilisation
• Electrical muscle stimulation
• Cycle ergometry
• Ongoing ambulatory rehab
Connally B, O’Neill B et al Thorax 2o16;0:1-10