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Rehabilitation in myopathies - dr venugopal kochiyil

Rehabilitation in Myopathies
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Rehabilitation in myopathies - dr venugopal kochiyil

  1. 1. Rehabilitation in myopathies Venugopal Kochiyil Medical Head of the Unit - Northern Adelaide Rehabilitation Service Modbury Hospital South Australia, Australia
  2. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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
  12. 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. 13. Issues to consider • Steroid induced myopathy • Steroid induced osteoporosis • Infections • Aspiration
  14. 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
  15. 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. 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. 17. Management • Falls prevention, assistive devices, AFOs • Immunomodulatory therapy ? • IVIgs • Swallow assessment • Nutritional support • Low resistance, endurance exercises • Passive ROM exercises
  18. 18. Prognosis • Tend to progress over time • Faster progression in elderly patients • Significant disability within 15 years of diagnosis
  19. 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. 20. Prognosis • Start recovering once statin is stopped • Usually recover within 6 months
  21. 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. 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. 23. Criteria for CIP • 1 and 2 criteria are the same • Electrophysiological evidence of axonal motor and sensory neuropathy • Absence of decremental response
  24. 24. Prognosis • 50% near complete recovery • Residual impairments in severely affected
  25. 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