4 Steps simple approach myopathy
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For the clinicopathoconference. Aim for teaching medical students to approach myopathy. Thank you Dr.Atiwat Soontornpun, MD. for his kindly advise
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4 Steps simple approach myopathy
- 1. NNC CMU The Northern Neuroscience Centre Chiang Mai University 4 simple steps Approach Myopathy 10 March 2016
- 2. Neuromuscularproblem CNS Sensory with/without motor Nerve Motor Muscle Constant weakness Proximal arms and legs Most of Myopathies Distal arm prox legs H. Myotonic dyst A. Inclusion body myositis Prox arms Distal legs H. FSHD Distal arms,legs H. Nonaka,Miyoshi Stiffness H. Myopathy w Myotonia A. Hypothyroid Episodic weakness Exercise triggered H. Metabolic Mitochondrial Myopathy Unrelated or delayed H.HypoKPP Oculobulbar Ptosis with Ophalmoplegia H. OPMD CPEO Ptosis without H. Myotonic dyst bulbar first H. OPMD LGMD 1A Infant onset H. Congenital NMJ Nerve Motor neuron
- 3. NNC CMU The Northern Neuroscience Centre Chiang Mai University Overview ⢠Step 1 : Symptom definition ⢠Step2 : Conceptual classification ⢠Step 3 : Pattern recognition ⢠Step4 : Investigation
- 4. NNC CMU The Northern Neuroscience Centre Chiang Mai University Step 1: Symptom definition Negative symptom ⢠Weakness ⢠Proximal ⢠Distal ⢠Bulbar ⢠Exercise intolerant/fatigue ⢠Muscle atrophy
- 5. NNC CMU The Northern Neuroscience Centre Chiang Mai University Positive symptom: ⢠Myalgia ⢠Muscle pain without contraction ⢠Cramp ⢠Episodic involuntary muscle contraction usually painful ⢠Myotonia ⢠Impaired relaxation after contraction ⢠Repeated -> improve (warm up phenomenon)
- 6. NNC CMU The Northern Neuroscience Centre Chiang Mai University Myopathy with myalgia ⢠Note : Not specific with inflammatory ⢠Infalmatory myopathies Dermatomyositis,Polymyositis ⢠Infectious myositis ⢠Mitochondrial myopathies ⢠Myeoadenylate deaminaase deficienccy ⢠Eosinophili-myalgia syndrome ⢠Tubular aggregate myopathy ⢠Becker dystrophy varient
- 9. Myotonia in simple physiology Muscle membrane hyperexcitability (repetitive repolarization cause delayed depolarization) 1. Na channel gain function 2. Cl channel loss function Berchtold MW et al.Physiol Rev 2000(80) 1215â1265
- 10. NNC CMU The Northern Neuroscience Centre Chiang Mai University Step 2: Conceptual classification Myopathy Hereditary Acquire 1. Structure 2. Channel 3.Metabolism
- 11. NNC CMU The Northern Neuroscience Centre Chiang Mai University Characters of hereditary myopathy 1. Slow progression (onset might be in adult) 2. Myotonia 3. Family history
- 12. Hereditarymyopathy Muscular dystrophy Congenital myopathy Channelopathies Metabolic myopathies Mitochondrial myopathy Myopathy with myotonia ⢠Glucose ⢠Lipid ⢠Myotonic dystrophy ( Cl channel) ⢠Duchene and Becker ⢠Emery-Drefuss ⢠Facioscapulo-humeral ⢠Limb-girdle ⢠Oculopaharyngeal ⢠myotonia congenital (Cl channel) ⢠Paramyotonia congenital (Na channel) ⢠HyperKPP (Na channel) ⢠HypoKPP (Ca channel) ⢠Anderson Tawil (K channel)
- 13. NNC CMU The Northern Neuroscience Centre Chiang Mai University Step 3 : Pattern recognition 1. Proximal arms and legs = Limb Girdle 2. Proximal arms and distal legs = Scapuloperoneal 3. Distal arms and proximal legs 4. Ptosis with or without ophthalmoparesis 5. Bulbar 6. Stiffness 7, 8, 9, 10 Carlyane E et al. Continuum 2013;19(6) 1674-97
- 14. ⢠Ptosis with Ophtalmoplegia ⢠OPMD : oculopharyngeal muscular dystrophy ⢠OPDM : coulopharyngodistal myopathy ⢠CPEO & Mitochondrial myopathy group ⢠Ptosis without ⢠Myotonic dystrophy ⢠Congential myopathy ⢠myofibrillar myopathy Ptosis with and without opthalmoplegia
- 15. Neuromuscularproblem CNS Sensory with/without motor Nerve Motor Muscle Constant weakness Proximal arms and legs Most of Myopathies Distal arm prox legs H. Myotonic dyst A. Inclusion body myositis Prox arms Distal legs H. FSHD Distal arms,legs H. Nonaka,Miyoshi Stiffness H. Myopathy w Myotonia A. Hypothyroid Episodic weakness Exercise triggered H. Metabolic Mitochondrial Myopathy Unrelated or delayed H.HypoKPP Oculobulbar Ptosis with Ophalmoplegia H. OPMD CPEO Ptosis without H. Myotonic dyst bulbar first H. OPMD LGMD 1A Infant onset H. Congenital NMJ Nerve Motor neuron
- 16. Distribution of weakness 10âs 30âs 50âs Age at the onset of symptom
- 17. DDx ⢠Hereditary ⢠Congenital myopathy ⢠Channelopathies ⢠Myopathy with myotonia ⢠Metabolic myopathy ⢠Mitochondrial myopathy ⢠Muscular dystrophy ⢠Duchene ⢠OPMD ⢠LGMD
- 18. NNC CMU The Northern Neuroscience Centre Chiang Mai University DDx ⢠Acquired ⢠Inflammatory ⢠Infection ⢠Drug/toxin ⢠Endocrine/nutrition ⢠Overused trauma
- 19. NNC CMU The Northern Neuroscience Centre Chiang Mai University Step 4: Investigation ⢠Total CK ⢠EMG ⢠Muscle biopsy ⢠Genetic testing
- 20. NNC CMU The Northern Neuroscience Centre Chiang Mai University Muscle biopsy
- 21. ⢠Rhabdomyolysis ⢠Alcohol :acute ⢠Cholesterol lowering drugs ⢠Toluene ⢠Drug abuse : amphetamine, heroine, cocain ⢠Inflammatory ⢠Levodopa ⢠Cimetidine ⢠D-penicilamine ⢠Procainamide Drugs induce myopathies that raise CK
- 22. ⢠Myosin loss ⢠Steroid ( involve type II muscle-> EMG also normal) ⢠Alcohol :chronic ⢠Nondepolarizing neuromuscular blocking ie. Tubercularin Drugs induce myopathies that NOT raise CK
- 23. NNC CMU The Northern Neuroscience Centre Chiang Mai University Alcoholic myopathy ⢠Acute âMost common cause of nontraumatic rhabdomyolysis âPatho: muscle fiber necrosis âDays after bing drinking âPrecipitate by hypoPO4 ie refeeding syndrome âRecovery days to wks after cessation of alc intake ⢠Chronic âCorelate with alcoholic cardiomyopathy âPatho: muscle fiber type II atrophy âRelate to high cumulative lifetime dose âRecovery 3-18 mos after cessation of alc intake
- 24. NNC CMU The Northern Neuroscience Centre Chiang Mai University Limb girdle muscular dystrophy : LGMD ⢠Definiton: genetic muscle disease with postnatal onset of progressive weakness affecting proximal upper and lower extremities ⢠Defect of gene required for muscle function ⢠AD form : 1A, 1BâŚ1H ⢠AR form : 2A, 2BâŚ2W
- 25. NNC CMU The Northern Neuroscience Centre Chiang Mai University
- 26. NNC CMU The Northern Neuroscience Centre Chiang Mai University Take home message Step 1 : Negative and positive symptoms Step 2 : Conceptual classification Hereditary vs Acquired Step 3 : Pattern recognition approach Distribution Proximal, distal, oculobulbar Pogression : Episodic, acute, chronic, nonprogress Step 4 : Investigation Serum CK , EMG, Muscle biopsy
- 27. NNC CMU The Northern Neuroscience Centre Chiang Mai University Reference ⢠ŕ¸ŕšŕ¸˛ŕ¸Łŕ¸˛ŕ¸ŕ¸Łŕ¸°ŕ¸Şŕ¸˛ŕ¸ŕ¸§ŕ¸´ŕ¸ŕ¸˘ŕ¸˛ŕ¸ŕ¸Ľŕ¸´ŕ¸ŕ¸´ŕ¸ . สลาŕ¸ŕ¸Ąŕ¸ŕ¸Łŕ¸°ŕ¸Şŕ¸˛ŕ¸ วิŕ¸ŕ¸˘ŕ¸˛ŕšŕ¸Ťŕšŕ¸ŕ¸ŕ¸Łŕ¸°ŕšŕ¸ŕ¸¨ŕšŕ¸ŕ¸˘ 2557 ⢠Carlyane E et al. Continuum 2013;19(6) 1674-97