Myopathies

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MYOPATHIES A SPECIAL AND SEPERATE ENTITY WITH SPECIFIC FEATURES IN EACH DISORDER MAKING US EASY FOR DIAGNOSIS,CONFIRMATION BY MUSCLE BIOPSY.THE SEMINAR WAS PRSENTED ON 06/07/2011...AT 09.00AM
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Myopathies

  1. 1. myopathies<br />DR.PRAVEENNAGULA<br />
  2. 2. UNVERRICHT<br />ERNST LEBERECHT WAGNER<br />ETIENNE JULES MAREY<br />HARVEY CUSHING<br />
  3. 3. Basic muscle anatomy.physiology<br />
  4. 4.
  5. 5. INTRODUCTION<br />MYOPATHY means primary skeletal muscle dysfunction.<br />WASTING--- related to muscle <br />MYALGIA– muscle pain<br />FIBRILLATIONS - when muscle fibers lose contact with their innervating axon producing a spontaneous action potential, "fibrillation potential" that results in the muscle fiber's contraction. not visible under the skin and are detectable through needle electromyography (EMG) and ultrasound.-pathological<br />FASCICULATIONS – visible spontaneuos contractions. <br />SPASM/CRAMP – sudden PAINFUL involuntary contraction of muscle<br />TONE – continuous and partial contraction of the muscles AT REST.resting muscle tension<br />CLONUS –series of involuntary muscle contractions.<br />MYOKYMIA – INVOLUNTARY ,groups of fasciculations –continuous undualtions of muscle.<br />
  6. 6. NERVOUS SYSTEM EXAMINATION<br /><ul><li>In motor unit points in favour of myopathy:
  7. 7. Muscle appearance – wasting ,atrophy (neurogenic)
  8. 8. ABSENT fasciculations (+Denervation)
  9. 9. Tenderness on palpation
  10. 10. Tone –normal ,decreased in advanced cases.
  11. 11. Distribution of weakness –proximal,distal (distal myopathies)
  12. 12. Pronator drift test
  13. 13. Tendon reflexes – normal /hypoactive in adv.cases
  14. 14. Babinski sign negative
  15. 15. SENSORY system is normal.
  16. 16. GAIT – lordosis on stance,increased on toe walking
  17. 17. Waddling gait – b/l pelvic girdle weakness
  18. 18. Genurecurvatum –quadriceps weakness.</li></li></ul><li>TYPE OF WEAKNESS<br />
  19. 19. APPROACH OF A PATIENT WITH INTERMITTENT WEAKNESS<br />
  20. 20. Approach to a patient with PERSISTENT WEAKNESS <br />
  21. 21. Based on other complaints<br />
  22. 22. EMG –assess the function of type I fibersnormal in steroid,disuse atrophy<br />
  23. 23. CLASSIFICATION<br /><ul><li>1.INFLAMMATORY /INFECTIOUS MYOPATHIES
  24. 24. 2.CONGENITAL DISTAL MYOPATHIES
  25. 25. 3.DISORDERS OF MUSCLE MEMBRANE EXCITABILITY
  26. 26. 4.DRUG INDUCED/TOXIN INDUCED MYOPATHY
  27. 27. 5.METABOLIC MYOPATHY
  28. 28. 6.ENDOCRINE MYOPATHY
  29. 29. 7.CRITICAL ILLNESS MYOPATHY
  30. 30. 8.MITOCHONDRIAL MYOPATHIES
  31. 31. 9.MUSCULAR DYSTROPHIES.
  32. 32. 10.MISCELLANEOUS</li></li></ul><li>INFLAMMATORY MYOPATIHES<br />Largest group of acquired and potentially treatable causes of skeletal muscle weakness .<br />POLYMYOSITIS (PM)<br />DERMATOMYOSITIS (DM)<br />INCLUSION BODY MYOSITIS (IBM)<br />1 :1,00,000 <br />PM – as alone is a rare disease affecting ADULTS.<br />DM – affects both children,adults W>M<br />IBM – M:F –3:1 ,caucasians,>50yrs.<br />
  33. 33. PATHOGENESIS<br /><ul><li>Autoimmune etiology - assosciation with other autoimmune CTD ,various Auto Ab, MHC ,T CELL myotoxicity,complement, response to immunotherapy.
  34. 34. AUTOANTIBODIES AND IMMUNOGENETICS:
  35. 35. 20% cases – ANA,anticytoplasmic antigens
  36. 36. Anticytoplasmic – anti RNP(anti synthetase),
  37. 37. Anti jo 1 – 75% cases -80% assosciation with ILD.
  38. 38. Anti Jo 1 –ILD ,RP,non erosive arthritis,MHCDR3,DRw52.
  39. 39. DRB1 *0301,DQB1*0201 --- 75% PM,IBM
  40. 40. DQA1*0501 –juvenile DM </li></li></ul><li>IMMUNOPATHOGENIC MECHANISMS<br /><ul><li>DERMATOMYOSITIS:
  41. 41. humoral immune mechanisms – microangiopathy.
  42. 42. Endomysial inflammatory infiltrates -B cells
  43. 43. auto AB -- complement pathway – C5b-9 membranolytic complex- release of proinflammatory cytokines – VCAM1,ICAM 1 on endothelial cells – migration of lymphoid cells to perimysial and endomysial spaces---necrosis,microinfarcts.
  44. 44. Remaining capillaries dilated due to ischemia
  45. 45. Perifascicular atrophy due to endofascicularhypoperfusion –periphery of muscle fascicles.</li></li></ul><li>
  46. 46. IMMUNOPATHOGENIC MECHANISMS<br /><ul><li>POLYMYOSITIS,IBM :
  47. 47. T cell mediated cytotoxicity
  48. 48. CD8 T cells,macrophages –invade and destroy MHC 1 muscle fibres(absent usually )
  49. 49. CD8/MHC 1 complex is characteristic of PM,IBM
  50. 50. These cells have perforin,granzyme granules –myonecrosis.
  51. 51. Antigen driven T cell response (auto AB in DM )
  52. 52. Antigens – endogenous (muscle),exogenous (viral)
  53. 53. Co stimulatory molecules are upregulated . </li></li></ul><li>ROLE OF NON IMMUNE FACTORS<br /><ul><li>IBM :
  54. 54. Degenerative process in addition to autoimmune process.
  55. 55. B amyloid deposits within vacuolated muscle fibres,cyto ox –ve mitochondria.
  56. 56. Amyloid is APP,chymotrypsin,apoE,tau
  57. 57. ?directly pathogenic /secondary
  58. 58. MHC I upregulation – ER stress – accumulation of misfoldedglycoproteins ,NFkB –cytokine activation .</li></li></ul><li>Association with viral infections<br /><ul><li>Coxsackie,influenza,paramyxoviruses,mumps,CMV,EBV.
  59. 59. Autoimmune myositis in coxsackie virus
  60. 60. Molecular mimicry of Anti RNAsynthetase and genomic RNA .
  61. 61. Best evidence of viral connection in PM,IBM –retroviruses.
  62. 62. HIV,HTLV 1 – PM,IBM
  63. 63. May be the initial manifestation or later of viral infection.
  64. 64. Retroviral antigens –endomyosial macrophages ,but not in muscle fibres.
  65. 65. AZT induced myopathy –mitochondrial – ragged red fibres.
  66. 66. AZT inhibits gamma DNA polymerase. </li></li></ul><li>Clinical features<br />In general :<br /><ul><li>PROGRESSIVE AND SYMMETRIC MUSCLE weakness (IBM –asymmetric).
  67. 67. Increasing difficulty with tasks – getting up from chair,climbingsteps,liftingobjects,combing hair.
  68. 68. Occular muscles are spared –even in advanced ,untreated cases –if involved ? Diagnosis
  69. 69. Head drop sign
  70. 70. dysphagia
  71. 71. Respiratory muscles involved in advanced cases.
  72. 72. Muscle wasting in untreated cases .
  73. 73. Sensation always normal .
  74. 74. DTR preserved.</li></li></ul><li>POLYMYOSITIS<br /><ul><li>Subacute inflammatory myopathy affecting adults,rarely children.
  75. 75. Facial muscles are unaffected
  76. 76. Diagnosis by exclusion.
  77. 77. As an isolated entity it is rare.
  78. 78. Commonly occurs in assosc. With systemic autoimmune </li></ul> disease ,viral,bacterial.<br /><ul><li>Drugs –AZT,D penicillamine</li></li></ul><li>DERMATOMYOSITIS<br /><ul><li>Characteristic rash accompanying/preceding muscle weakness. – increased photosensitivity
  79. 79. Blue purple discoloration on the upper eyelids with edema.(heliotrope rash)
  80. 80. A flat red rash on the face and upper trunk.
  81. 81. Erythema of the knuckles with a raised violaceous scaly eruption – gottron’s sign .
  82. 82. Erythematous rash on anterior chest – V sign.
  83. 83. Back and shoulders – shawl sign .
  84. 84. Dilated capillary loops at the base of the finger nails.
  85. 85. Mechanic’s hands –irregular dirty horizontal lnies on the palmar surface of fingers.
  86. 86. Muscle tenderness,myalgia –connective tissue disease.
  87. 87. Dermatomyositis sine myositis– muscle strength normal
  88. 88. Perivascularperimysial inflammation </li></li></ul><li>
  89. 89. INCLUSION BODY MYOSITIS<br /><ul><li>>50 yrs of age,most common inflammatory myopathy.
  90. 90. Often misdiagnosed as PM
  91. 91. Suspected when doesnot respond to therapy.
  92. 92. Weakness and atrophy of distal muscles,footextensors,deep finger flexors –all cases –clue to early diagnosis.
  93. 93. Fine motor movements –affected early
  94. 94. Falling is common – quadriceps –buckling of knees.
  95. 95. DTR –depressed in presence of atrophy .
  96. 96. Dysphagia -60% cases
  97. 97. Slow and steady progression
  98. 98. Assisted devices required</li></li></ul><li>Familial IBM<br /><ul><li>20 % CASES -systemic autoimmune or connective tissue disease.
  99. 99. Familial IBM
  100. 100. Hereditary IBM - heterogenous group of recessive,dominant inherited syndromes,non inflammatory
  101. 101. May spare the quadriceps –iranian JEWS
  102. 102. Chromosome 9p1
  103. 103. Mutations in the UDP – N acetylglucosamine 2- epimerase n –acetylmannosaminekinase GNE gene.</li></li></ul><li>
  104. 104.
  105. 105. Associated clinical findings <br /><ul><li>EXTRAMUSCULAR MANIFESTATIONS:
  106. 106. Systemic symptoms --- fever,malaise -- CTD.
  107. 107. Joint contractures –DM in children
  108. 108. Dysphagia ,GIabn – DM ,IBM
  109. 109. Cardiac disturbances –arryhthmias,DCM,CCF
  110. 110. Pulmonary dysfunction – ILD,drug induced – t RNA ab – 10%
  111. 111. Subcutaneous calcifications –DM
  112. 112. Arthralgias,deformingarthropathy – anti jo1 ab
  113. 113. MALIGNANCIES– DM>PM,IBM
  114. 114. Nasopharyngeal cancer in india
  115. 115. Ovarian,breast,melanoma ,colon,NHL
  116. 116. OVERLAP SYNDROMES – DM – SSc,PM – RA ,sjogren’s,SLE
  117. 117. Anti PM/Scl - overlap syndrome of DM.</li></li></ul><li>DIFFERENTIAL DIAGNOSIS<br /><ul><li>SUBACUTE OR PROGRESSIVE MUSCLE WEAKNESS :
  118. 118. SPINAL MUSCULAR ATROPHY,ALS
  119. 119. UMN signs ,denervation signs on EMG
  120. 120. MUSCULAR DYSTROPHIES – progress over years ,rarely present >30 yrs.
  121. 121. RAPIDLY ADVANCING MUSCULAR dystrophy –fascioscapulohumeral,dysferlin –inflammatory cell infiltration early.
  122. 122. Trial of glucocorticoids,MHC/CD8
  123. 123. MYOPHOSPHORYLASE /ACID MALTASE deficiency
  124. 124. ENDOCRINE
  125. 125. TUMOR ASSOCIATED CACHEXIA
  126. 126. LAMBERT EATON SYNDROME,MG</li></li></ul><li><ul><li>ACUTE MUSCLE WEAKNESS:
  127. 127. GBS
  128. 128. Transverse myelitis
  129. 129. Poliomyelitis
  130. 130. Myophosphorylase deficiency
  131. 131. PARASITIC polymyositis.
  132. 132. Tropical polymyositis –s.aureus
  133. 133. Periodic paralysis
  134. 134. Chronic ALCOHOLICS
  135. 135. MYOFASCITIS – skin induration -eosinophilicmyofascitis
  136. 136. NECROTIZING MYOSITIS – CK is high.ILD,CMP,anti SRP.
  137. 137. DRUG INDUCED – d penicillamine
  138. 138. Weakness due to muscle pain ,tenderness –PMR -type ii muscle fibres,CFS –biopsy normal. </li></li></ul><li>LAB INVESTIGATIONS<br /><ul><li>1.SERUM MUSCLE ENZYMES :most sensitive CK .
  139. 139. Parallels disease activity (active -50 fold)
  140. 140. Normal when CTD +PM,DM
  141. 141. serumGOA,GPT,LDH,aldolase – elevated.
  142. 142. 2.EMG – short duration,low amplitude polyphasic units on voluntary activation,spontaneuousfibrillations,positive sharp waves.
  143. 143. Mixed potentials –IBM
  144. 144. Identifies acute/chronic,excludesnuerogenic
  145. 145. 3.MRI –muscle biopsy
  146. 146. 4.muscle biopsy – definitive test
  147. 147. Inflammation is the hallmark </li></li></ul><li>Muscle biopsy<br /><ul><li>POLYMYOSITIS –
  148. 148. Primary – T cell infiltrates (endomyosial) surround healthy muscle fibers- phagocytosis.
  149. 149. CD8/MHC 1 –confirms the diagnosis.
  150. 150. Alkaline phosphatase –react positively.
  151. 151. DERMATOMYOSITIS – perivascular,interfascicularseptae
  152. 152. Intramuscular BV – endothelial hyperplasia
  153. 153. Wedge shaped microinfarcts.
  154. 154. Presence of perifascicular atrophy is diagnostic of DM in absence of inflammation also.
  155. 155. INCLUSION BODY MYOSITIS – rimmed vacuoles,fatrepalcement,ragged red fibres,amyloid deposits. </li></li></ul><li>
  156. 156. TREATMENT<br /><ul><li>GOAL : improve muscle strength,ameliorateextramuscular manifestations.
  157. 157. Discontinue the drugs if after trial no improvement of muscle strength.
  158. 158. 1.GLUCOCORTICOIDS :oral prednisolone –high doses – 1mg/kg/day – 3-4 weeks,taper later 10 weeks – 1mg/kg every other day.
  159. 159. Lowest possible dose to be used.
  160. 160. Efficacy by third month
  161. 161. DM > PM in response.
  162. 162. Steroid myopathy – increased weakness –2- 8 weeks.</li></li></ul><li>Treatment cont…<br /><ul><li>2.IMMUNOSUPPRESSIVE THERAPY :
  163. 163. 75% patients ultimately require.
  164. 164. A.AZATHIOPRINE : 3mg/kg/day
  165. 165. B.MTX : 7.5 mg weekly for the first 3 weeks -2.5mg/wk- 25 mg/wk --- MTX pneumonitis.
  166. 166. C.mycophenolate MOFETIL – 2.5 mg/d
  167. 167. D.rituximab - IN DM
  168. 168. E.CYCLOSPORINE – inconsistent benefit
  169. 169. F.TACROLIMUS - PM difficult cases.
  170. 170. 3.IMMUNOMODULATION:
  171. 171. IVIg in refractory DM -short term benefit
  172. 172. 2g/kg over 2-5 days /course
  173. 173. PLASMAPHERESIS,LEUKOPHERESIS - NOT EFFECTIVE IN PM,DM</li></li></ul><li>EMPIRICAL APPROACH IN TREATMENT<br /><ul><li>STEP 1 – HIGH DOSE PREDNISOLONE</li></li></ul><li>NO RESPONSE TO TREATMENT<br /><ul><li>IBM or metabolic myopathy,musculardystrophy,endocrinopathy.
  174. 174. Repeat muscle biopsy
  175. 175. Calcinosis of DM –difficult to treat
  176. 176. IBM –resistant to immunosupressive therapy
  177. 177. Drugs not to be withdrawn
  178. 178. IVIg and prednisolone – not effective
  179. 179. PROGNOSIS -5 yr surivival – 95%,10 yr -85%
  180. 180. Death –pulmonary,cardiac
  181. 181. Worse prognosis – older,severemainfestations at prsentation
  182. 182. DM responds favorably than PM,IBM
  183. 183. IBM –least favorable prognosis.</li></li></ul><li>ENDOCRINE MYOPATHIES<br /><ul><li>Muscle fatigue is more common than true weakness.
  184. 184. Serum CK LEVELS are normal.
  185. 185. Muscle histology –atrophy rather than destruction.
  186. 186. All respond to treatment
  187. 187. THYROID DISORDERS:
  188. 188. HYPOTHYROIDISM –proximal muscle weakness-1/3
  189. 189. Slow muscle contraction and relaxation -25% cases.
  190. 190. Relaxation phase prolonged –ankle,biceps reflex
  191. 191. Serum CK 10 times normal,EMG normal
  192. 192. Muscle may be enlarged?,biopsy –no features</li></li></ul><li><ul><li>HYPERTHYROIDISM:
  193. 193. Proximal muscle weakness,atrophy of muscles.
  194. 194. DTRs enhanced response.
  195. 195. Bulbar,esophageal,respiratory muscles affected.
  196. 196. Fasciculations+DTRs –ALS misdiagnosis
  197. 197. Acquired hypokalemic periodic paralysis,MG,gravesophthalmopathy
  198. 198. Serum CK levels are normal
  199. 199. EMG is normal
  200. 200. Muscle histology –atrophy of muscles</li></li></ul><li><ul><li>CHRONIC THYROTOXIC MYOPATHY – nodular goitre.
  201. 201. Men>women.
  202. 202. Basedow paraplegia.
  203. 203. Atrophy – shoulder and hand muscles.
  204. 204. Tremor twitches but not fasciculations
  205. 205. GRAVES OPTHALMOPATHY- IR,MR,strabismus,diplopia
  206. 206. Infiltrative opthalmopathy
  207. 207. THYROTOXIC HPP- not familial,fewhours,adult life.
  208. 208. Hypothyroidism aggravates the weakness of MG
  209. 209. Kocher -debresemelgainesyndrome,hoffmann syndrome</li></li></ul><li>Adrenal disorders<br /><ul><li>Most commonly diagnosed endocrine muscle disease.
  210. 210. Proximal muscle weakness.
  211. 211. Muscle wasting –striking feature+ cushingoidfacies
  212. 212. Type 2b fibres atrophy on histology
  213. 213. Potassium depletion –CONN’S syndrome –NM complications.
  214. 214. Serum CK LEVELS ELEVATED,vacuoles on biopsy.
  215. 215. Vitamin D deficiency
  216. 216. Parathyroid disorders –hypo,hyper
  217. 217. PITUITARY DISORDERS
  218. 218. DIABETES MELLITUS—THIGH muscles,pain tenderness in thigh</li></ul> Hard induratedmuscle,vastuslateralis<br />
  219. 219. CRITICAL ILLNESS MYOPATHY<br /><ul><li>Acute quadriplegic myopathy/acute steroid myopathy
  220. 220. In cases of severe asthma ,sepsis and shock.
  221. 221. Neuromuscular blocking agents in 80% cases.
  222. 222. High doses of corticosteroids 1mg/kg ,pancuronium -500-4,000 mg
  223. 223. Difficulty in weaning from the ventilator
  224. 224. Tendon relfexes are normal- diminished –polyneuropathy
  225. 225. Serum CK levels elevated.
  226. 226. EMG –MYOPATHY.type 2 fibers vacuolation.
  227. 227. Striking loss of myosin filaments
  228. 228. Severe cases– myoglobinuria,renal failure.
  229. 229. Denervation of muscles –increase in glucocorticoid receptors
  230. 230. Recovery over 6- 12 weeks</li></li></ul><li>CARCINOMATOUS MYOPATHY<br /><ul><li>Syndromes of NMJ – LAMBERT EATON SYNDROME,MG
  231. 231. Syndromes of muscle – polymyositis /dermatomyositis,acute necrotizing myopathy.
  232. 232. ACUTE NECROTIZING MYOPATHY-rhabdomyolysis
  233. 233. Painful myopathy
  234. 234. Necrosis of muscle– raised CK,myoglobinuria
  235. 235. Urine is cola coloured .
  236. 236. Detected by radioimmunoassay
  237. 237. Alkalinization of urine –nahco3
  238. 238. Mannitol,diuretics,IV fluids</li></li></ul><li>DRUG INDUCED MYOPATHY<br /><ul><li>MYOPATHY FROM LIPID LOWERING AGENTS
  239. 239. GLUCOCORTICOID RELATED MYOPATHIES
  240. 240. MYOPATHY OF NON DEPOLARIZING NEUROMUSCULAR BLOCKING AGENTS
  241. 241. DRUG INDUCED MITOCHONDRIAL MYOPATHY
  242. 242. DRUGS OF ABUSE AND RELATED MYOPATHY
  243. 243. DRUG INDUCED AUTOIMMUNE MYOPATHIES
  244. 244. OTHER </li></li></ul><li>
  245. 245. DISORDERS OF MUSCLE MEMBRANE EXCITABILITY<br />
  246. 246. <ul><li>POTASSIUM CHANNEL DISORDERS:
  247. 247. 1.ANDERSEN TAWIL SYNDROME:
  248. 248. Episodic weakness,dysmorphic features.
  249. 249. cardiac arrythmias are life threatening.
  250. 250. autosomal dominant –kir 2.1 gene
  251. 251. Acetazolamide
  252. 252. CHLORIDE CHANNEL DISORDERS:
  253. 253. AD –THOMSEN’S DISEASE
  254. 254. AR – BEKER’S DISEASE
  255. 255. Myotonia worsened by cold,improved by activity
  256. 256. Muscle strength normal in thomson’s
  257. 257. Quinine,mexiletine,phenytoin.</li></li></ul><li>DISTAL MYOPATHIES<br />****LIMITED TO SKELETAL MUSCLES<br />
  258. 258. CONGENITAL MYOPATHIES<br /><ul><li>Specific histochemical and structural abn in muscle.</li></li></ul><li>METABOLIC MYOPATHIES<br />
  259. 259. Lipid abnmyopathies<br />
  260. 260. MITOCHONDRIAL MYOPATHIES<br /><ul><li>TERM RAGGED RED FIBRES –ABNORMAL MITOCHONDRIA ON MODIFIED TRCIHROME STAIN .
  261. 261. Mitochondria are enlarged,bizarrelyshaped,crystalline inclusions
  262. 262. Most common is CPEO >50% cases of mitochondrial myopathies.</li></li></ul><li>WHITE TRICHROME STAIN<br />Type I fiber atpASE STAIN<br />
  263. 263. Pure myopathy syndromes<br /><ul><li>Affects only the muscle
  264. 264. Recurrent myoglobinuria without fixed weakness and thus resemble a glycogen storage or CPT deficiency.
  265. 265. MITOCHONDRIAL DNA DEPLETION MYOPATHY :
  266. 266. Clinically indistinguishgable from muscular dystrophy.
  267. 267. Simulates DMD
  268. 268. Seizures,CMP may be present.
  269. 269. Serum CK 20-30 times normal,EMG –ragged red fibres.
  270. 270. AR condition
  271. 271. TK2 gene mutation 16 q22—supplies deoxyribonucleotides.</li></li></ul><li>MISCELLANEOUS<br /><ul><li>NEUROMYOTONIA –CONTINUOUS MUSCLE FIBER ACTIVITY.
  272. 272. Hyperexcitability of terminal motor neuron plates.
  273. 273. Rippling of muscle fibers – myokymia –main clinical sign.
  274. 274. Walking is laborious –armadillo syndrome.
  275. 275. Muscle activity during sleep.
  276. 276. Curare abolishes.
  277. 277. ISAAC syndrome</li></li></ul><li>TRIALS<br /><ul><li>827 STUDIES ON MYOPATHIES
  278. 278. MOLECULAR AND GENETIC STUDIES OF CONGENITAL MYOPATHIES.
  279. 279. PHARMACOKINETIC STUDY ON N ACETYL NEURAMINIC ACID
  280. 280. CHARACTERIZATION OF IBM WITH PAGET’S,FTD
  281. 281. IS MYOPATHY PART OF STATIN THERAPY –IMPOSTER 16
  282. 282. STEM CELL TRANSPLANTATION IN IDIOPATHIC INFLAMMATORY MYOPATHY DISORDERS
  283. 283. ANAKINRA IN MYOSITIS
  284. 284. THE EFFECT OF STATINS ON MUSCLE PERFORMANCE –STOMP STUDY
  285. 285. METHIMAZOLE FOR DERMATOMYOSITIS
  286. 286. ARIMOCOLOL IN SPORADIC INCLUSION BODY MYOSITIS
  287. 287. LOG ON TO www.clinicaltrials.gov </li></li></ul><li>TAKE HOME MESSAGE<br /><ul><li>MYOPATHIES need to be categorised based on the pattern of weakness intermittent,persistent.
  288. 288. Usually present as proximal muscle weakness.
  289. 289. The clinical examination reveals wasting>atrophy,DTR being normal.
  290. 290. Laboratory wise raised CK levels normally.
  291. 291. Confirmatory diagnosis by MUSCLE BIOPSY .EMG –normal.
  292. 292. POLYMYOSITIS is usually seen in assosciation with other disorders .diagnosis by exclusion.
  293. 293. DERMATOMYOSITIS responds well to treatment.
  294. 294. IBM can be misdiagnosed as PM
  295. 295. Drug induced myopathy --- check the list for statins,steroids.
  296. 296. Critical illness myopathy,neuropathy –increasing entity.
  297. 297. Congenital distal myopathies,mitochondrial to be considered in D.D </li></li></ul><li>THANK YOU<br />

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