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Rehabilitation in myopathies - dr venugopal kochiyil
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 â 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 â myogenic, arthrogenic or soft tissue
⢠High risk in dystrophinopathies
⢠To prevent contractures â 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%) â 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 â 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 â Azothiaprine, MTX
⢠IVIg â def role in resistant and recurrent presentation
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 â rimmed vacuoles, mononuclear
inflammatory cells invading non necrotic muscle
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
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