Neuropathies & myopathies - an overview

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This ppt is an overview of Neuropathies and myopathies..and from the physiologist view..hope will be useful template!

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Neuropathies & myopathies - an overview

  1. 1. Neuropathies & Myopathies Dr. Dinesh T,11/19/2011 Jipmer physiologist Junior resident 1
  2. 2. Neuropathies11/19/2011 Jipmer physiologist 2
  3. 3. Definition Damage to nerves which may be caused either by diseases or trauma to the nerve or as a component of systemic illness11/19/2011 Jipmer physiologist 3
  4. 4. • The neuropathy is a symptom of another disorder• In most common forms of polyneuropathy, the nerve fibers most distant from the brain and the spinal cord malfunction first.• Pain and other symptoms often appear symmetrically11/19/2011 Jipmer physiologist 4
  5. 5. • The peripheral nerves include: Cranial nerves (with the exception of the second) Spinal nerve roots Dorsal root ganglia Peripheral nerve trunks and their terminal branches Peripheral autonomic nervous system11/19/2011 Jipmer physiologist 5
  6. 6. Symptoms in neuropathyA wide array of symptoms can occur when nerves are damagedo Paresthesiao Sensitivity to touch,  Positive Pins and needles Tingling Burning Negative Numbness Deadness As if wearing shocks and walk11/19/2011 Jipmer physiologist 6
  7. 7. In chronic course symptoms worse, muscle wasting, paralysis, or gland dysfunction11/19/2011 Jipmer physiologist 7
  8. 8. Neuropathy - Signs• Distal sensory loss• Distal weakness and atrophy• Decreased or absent reflexes – Ankle jerks lost first11/19/2011 Jipmer physiologist 8
  9. 9. Various classifications11/19/2011 Jipmer physiologist 9
  10. 10. Etiological Classification of neuropathiesHereditary Neuropathies • Acquired metabolic and toxic – Hereditary motor and sensory neuropathies neuropathy – type I – Peripheral neuropathy in adult – HMSN – Type II onset Diabetes – Dejerine – Sottas Neuropathy – Metabolic and nutritional HMSN- type III peripheral neuropathies – HSMN – type IV – Neuropathies associated with malignancy – HSMN – type V – Toxic neuropathies • Traumatic neuropathiesInflammatory neuropathies• Immune mediated o Guillain-Barré syndrome o Chronic inflammatory demyelinating polyradiculoneuropathy• Infectious – Leprosy – Diphtheria – Varicella – zoster11/19/2011 Jipmer physiologist 10
  11. 11. Medications Causing Neuropathieso Axonal o Demyelinating Amiodarone Vincristine Chloroquine Paclitaxel Suramin Nitrous oxide Gold Colchicine Isoniazid Hydralazine o Neuronopathy Metronidazole Thalidomide Pyridoxine Cisplatin Didanosine Lithium Pyridoxine Alfa interferon Dapsone Phenytoin Cimetidine Disulfiram Chloroquine Ethambutol Amitriptyline11/19/2011 Jipmer physiologist 11
  12. 12. Pathophysiological classification• Motor , sensory, or autonomic• Mononeuropathy , polyneuropathy or mononueritis multiplex• Focal, multifocal or symmetric• Proximal or distal• Axonal, demyelinating or both• Acute, sub acute or chronic11/19/2011 Jipmer physiologist 12
  13. 13. • Some neuropathies may affect all three types of nerves, others primarily affect one or two types. • Predominately motor neuropathy • Predominately sensory neuropathy • Sensory-motor neuropathy • Autonomic neuropathy• Impaired function and symptoms depend on the type of nerves that are damaged.11/19/2011 Jipmer physiologist 13
  14. 14. • Mononeuropathy involve damage to only one nerve• When multiple nerves supplying one limb are affected-called polyneuropathy.• Two or more isolated nerves in separate areas of the body are affected-called mononeuritis multiplex11/19/2011 Jipmer physiologist 14
  15. 15. o Focal neuropathies include common compressive neuropathies such as carpal tunnel syndrome, ulnar neuropathy ,peroneal neuropathy o Multifocal neuropathy suggests a mononeuritis multiplex that may be caused, for example, by vasculitis or diabetes11/19/2011 Jipmer physiologist 15
  16. 16. Axonal degeneration• Primary destruction of the axon with secondary degeneration of its myelin sheath• Generalized abnormality in the neuron cell body- neuronopathy• Abnormality in the axon - axonapathy11/19/2011 Jipmer physiologist 16
  17. 17. Segmental demyelinationDysfucntion of Schwann cell or damage to the myelin sheath Denuded axon provide signal for remyelination Precursor cells within endoneurium replace injured cells Cells proliferate and engulf axon.= remyelination in time 11/19/2011 Jipmer physiologist 17
  18. 18. Neurophysiological classification• Uniform demyelinating sensorimotor poly neuropathy• Segmental demyelinating, motor more than sensory neuropathy• Axonal , motor more than sensory polyneuropathy• Axonal sensory polyneuropathy• Axonal mixed sensorimotor polyneuropathy• Mixed axonal and demyelinating sensorimotor polyneuropathy11/19/2011 Jipmer physiologist 18
  19. 19. Uniform demyelinating sensorimotor poly neuropathy• Hereditary motor sensory neuropathy- type I, III,IV• Leucodystrophies• Tangier disease• Cockayne syndrome• Congenital cerebrotendinous xanthomatosis• Congenital hypomyelinating neuropathies11/19/2011 Jipmer physiologist 19
  20. 20. HMSN I (Charcot- Marie- Tooth I)• HSMN I – AD is the most common hereditary neuropathy.• CMT-I A chromosome 17p11 , CMT-IB chromosome 1q22 , CMT-IC 16p13, chromosome , CMT-IX chromosome Xq13.1• Slowly progressive distal weakness• Foot deformity, areflexia , distal sensory loss• Upper limb ataxia, tremor, peripheral n hypertrophy11/19/2011 Jipmer physiologist 20
  21. 21. Neurophysiological features• Conduction velocity less than 25% of lower limit• Median motor forearm conduction< 38 m/s• Uniform NCV changes in adjacent nerves• Absence of conduction block and temporal dispersion• F response• Needle EMG shows minimal fibrillations in distal muscles.11/19/2011 Jipmer physiologist 21
  22. 22. Uniform demyelinating sensorimotor poly neuropathy-Electrophysiological studies ( NCS ) showo Uniform slowing of NCVo Similar NCV slowing in adjacent nerveso Absence of conduction block and temporal dispersiono Prolongation of F response commensurate with NCV slowing11/19/2011 Jipmer physiologist 22
  23. 23. Segmental demyelinating motor more than sensory neuropathyo Acute inflammatory demyelinating poly radiculo neuropathy AIDPo Chronic inflammatory demyelinating poly radiculo neuropathy CIDPo Multifocal motor neuropathyo Paraproteinemiao HIV neuropathyo Lyme diseaseo Diphtheriao Penicillamine11/19/2011 Jipmer physiologist 23
  24. 24. AIDPo The prototypeo Distal paresthesia with symmetric weaknesso Distal areflexiao Variants are pure motor, pure sensory, autonomic, relapsing, and Miller fisher typeso Cranial nerves esp facial n and bulbar may be involvedo Respiratory muscles are severely involved in about 2511/19/2011 Jipmer physiologist 24
  25. 25. 11/19/2011 Jipmer physiologist 25
  26. 26. Pathophysiology of GBS• Pathological findings include inflammatory and demyelinating changes.• Monocytes and macrophages appear to attack myelin sheaths.• Myelinated fibers show segmental demyelination during the first few days. Segmental remyelination occurs subsequently.• The lesions have a perivenular distribution11/19/2011 Jipmer physiologist 26
  27. 27. Chronic inflammatory demyelinating polyneuropathy• Chronic progressive or relapsing neuropathy, motor > sensory.• Electrophysiology: slow conduction velocity & conduction block• Pathology: segmental demyelination and remyelination, onion bulbs, fibrosis and little or no lymphocytic infiltration of tissue.11/19/2011 Jipmer physiologist 27
  28. 28. Segmental demyelinating motor more than sensory neuropathyNerve conduction studieso Slowing of motor and sensory conduction velocityo Prolongation of terminal latencyo Conduction blocko Dispersion and prolonged or absent F waves11/19/2011 Jipmer physiologist 28
  29. 29. Axonal, motor more than sensory neuropathy• Axonal type of GBS• Acute intermittent porphyria• HSMN type II , V• Toxic neuropathies such as lead, dapsone• Paraneoplastic syndrome• Metabolic – hypoglycemia• Critical care neuropathy11/19/2011 Jipmer physiologist 29
  30. 30. • Distal symmetric weakness and wasting with minimal sensory loss11/19/2011 Jipmer physiologist 30
  31. 31. Axonal,motor more than sensory neuropathy• Nerve conduction studies• Reduced CMAP amplitude• NCV is normal• SNAP amplitudes are also decreased• Fibrillations appear in distal muscles11/19/2011 Jipmer physiologist 31
  32. 32. Sensory axonal polyneuropathy• Diabetic neuropathy• Carcinomatous sensory neuropathy• HSMN type I- IV• Friedrich ataxia• Abetalipoproteinemia• Toxins - cisplatin• Pyridoxine overdosage• Vt –E neuropathy• Malabsorption• Acromegaly,11/19/2011 Jipmer physiologist 32
  33. 33. Diabetic neuropathy• Onset of neuropathy depends upon the duration of illness• 50% diabetics have peripheral neuropathy of which 80% have had the illness for >15 years• Distal symmetric sensory or sensorimotor, autonomic, focal or multifocal asymmetric• Symmetric neuropathy involves distal sensory , motor nerves .• Decreased sensation, loss of pain sensation – ulcer11/19/2011 Jipmer physiologist 33
  34. 34. Diabetic neuropathy• Predominant pathology is axonal neuropathy.• In chronic cases segmental demyelination also seenPathophysiology –• Loss of small myelinated fibers and unmyelinated fibers. But large fibers can also be affected.• Endoneurial arterioles show thickening, hyalination, intense PAS positivity in the walls and extrensive reduplication basement membrane11/19/2011 Jipmer physiologist 34
  35. 35. Sensory axonal polyneuropathy• Nerve conduction studies• Diminished or absent SNAP amplitude in the setting of normal motor nerve conduction velocity11/19/2011 Jipmer physiologist 35
  36. 36. Axonal type of mixed sensorimotor neuropathy• Nutritional deficiencies (vitamin deficincy, alcoholism)• Metabolic ( diabetic, uraemia, liver disease, amyloidosis)• Connective tissue disorders (rheumatoid arthritis, SLE, PAN)• Multiple myeloma• Carcinoma• Cryoglobunemia• Heavy metals – lead, arsenic, gold, mercury• Drugs- metronidazole, phenytoin etc11/19/2011 Jipmer physiologist 36
  37. 37. • Paresthesia and dyesthesia of feet and distal legs• Wasting is marked• Loss of ankle reflex• Pathophysiology – evidence of degeneration of distal portion of axons11/19/2011 Jipmer physiologist 37
  38. 38. Axonal type of mixed sensorimotor neuropathyNerve conduction studies• Reduced or absent SNAP• CMAP amplitude decreases and motor conduction velocity also decrease in later stageEMG• Fibrillations and positive sharp waves are prominent in distal muscles.• Temporal dispersion on proximal stimulation is not found as in demyelinating neuropathies11/19/2011 Jipmer physiologist 38
  39. 39. Mixed axonal loss and demyelinating neuropathy• Diabetes• Uremia• Paraproteinemia11/19/2011 Jipmer physiologist 39
  40. 40. • Paresthesia, dyesthesia or numbness• Reduced vibration and two point discrimination• Pathophysiology – segmental demyelination and remyelination along with axonal degeneration11/19/2011 Jipmer physiologist 40
  41. 41. Mixed axonal loss and demyelinating neuropathy• Nerve conduction studies• Reduced or unrecordable CMAP, SNAP or both• Moderate to severe slowing of NCV with temporal dispersion of CMAP11/19/2011 Jipmer physiologist 41
  42. 42. Clinical examination• Thorough history and physical examination is needed.• Cranial nerve examination• Motor , sensory, autonomic nervous system examination• Fundus examination• Lymphadenopathy , hepatomegaly or splenomegaly, and skin lesions11/19/2011 Jipmer physiologist 42
  43. 43. Lab tests:• CBC, electrolytes, ESR• Fasting serum glucose, glycosylated hemoglobin, blood urea nitrogen, creatinine,• Liver , kidney,, thyroid function studies• Inflammatory markers,• Total protein level• Vit D, B12,• Cytology• CSF• Urinalysis• Nerve biopsy11/19/2011 Jipmer physiologist 43
  44. 44. Electrophysiologic studies• EMG and nerve conduction studies (NCS) are often the most useful initial laboratory studies in the evaluation of a patient with peripheral neuropathy• Confirm the presence of a neuropathy• Provide information as to the type of fibers involved (motor, sensory, or both), the pathophysiology (axonal loss versus demyelination) and a symmetric versus asymmetric or multifocal pattern of involvement.11/19/2011 Jipmer physiologist 44
  45. 45. Electrophysiologic studies• The limitations of EMG/NCS. – There is no reliable means of studying proximal sensory nerves. – NCS results can be normal in patients with small-fiber neuropathies – Lower extremity sensory responses can be absent in normal elderly patients.• EMG/NCS are not substitutes for a good clinical examination.11/19/2011 Jipmer physiologist 45
  46. 46. Treatment• The goal of treatment is to manage the underlying condition causing the neuropathy and repair damage, as well as provide symptom relief.11/19/2011 Jipmer physiologist 46
  47. 47. Treatment• Medical management – Analgesics . – antiepileptic drugs, including gabapentin, phenytoin, and carbamazepine – some classes of antidepressants, including tricyclics such as amitriptyline. – Mexiletine – local anesthetics such as lidocaine or topical patches containing lidocaine – Codeine/oxycodone11/19/2011 Jipmer physiologist 47
  48. 48. Treatment• Mechanical aids can help reduce pain and lessen the impact of physical disability. – Hand or foot braces can compensate for muscle weakness or alleviate nerve compression. – Orthopedic shoes can improve gait disturbances and help prevent foot injuries in people with a loss of pain sensation.• If breathing becomes severely impaired, mechanical ventilation can provide essential life support.11/19/2011 Jipmer physiologist 48
  49. 49. Treatment• Surgical intervention often can provide immediate relief from mononeuropathies caused by compression or entrapment injuries. – Repair of a slipped disk can reduce pressure on nerves where they emerge from the spinal cord; the removal of benign or malignant tumors can also alleviate damaging pressure on nerves. – Nerve entrapment often can be corrected by the surgical release of ligaments or tendons.11/19/2011 Jipmer physiologist 49
  50. 50. Myopathies11/19/2011 Jipmer physiologist 50
  51. 51. Definition Neuromuscular disorders in which the primary symptom is muscle weakness due to dysfunction of muscle fiber11/19/2011 Jipmer physiologist 51
  52. 52. Introduction• Worldwide incidence of all inheritable myopathies is about 14%• Overall incidence of muscular dystrophy is about 63 per 1 million.• Worldwide incidence of inflammatory myopathies is about 5–10 per 100,000 people. More common in women• Corticosteroid myopathy is the most common endocrine myopathy and endocrine disorders are more common in women• Incidence of metabolic myopathies – increasing11/19/2011 Jipmer physiologist 52
  53. 53. Myopathy: symptoms• Muscle pain and fatigue; exercise intolerance• Proximal and symmetric weakness – Waddling gait; difficulty of rising from sitting, climbing stairs; Gower’s sign – Hyperextension of the knee – Increased lordosis of the lumbar spine, scoliosis – Contractures, tight Achilles tendons• Myopathic face• Muscle atrophy; pseudohypertrophy• Myotonia• Tendon reflexes are normal or depressed11/19/2011 Jipmer physiologist 53
  54. 54. Clinical examination• Thorough clinical examination!• Observation – look for muscle atrophy, deformities• Strength testing• Functional testing – Stand up from a chair – Walk – Step up on a low stool• REFLEXES and SENSATION11/19/2011 Jipmer physiologist 54
  55. 55. Types of muscle diseases• Hereditary muscle diseases – Denervation atrophy – Muscle dystrophies – Muscle channelopathies – Mitochondrial myopathies – Metabolic myopathies• Acquired muscle diseases – Inflammatory myopathies – Endocrine and toxic myopathies – Infectious muscle diseases11/19/2011 Jipmer physiologist 55
  56. 56. Myopathic Disorders• Inflammatory Myopathies • Congenital myopathies – Polymyositis – Central core disease – Dermatomyositis – Nemaline myopathy – Inclusion body myositis – Myotubular – Viral – Fiber-type disproportion• Muscular dystrophies • Metabolic myopathies – Duchenne muscular – Glycogenoses – Limb-girdle – Mitochondrial – Congenital – Periodic paralysis – Fasioscapulohumeral • Endocrine myopathies – Oculopharyngeal – Thyroid – Emery – Dreifuss – Parathyroid – Distal (Welander) – Adrenal/steroid• Myotonic Syndromes – Pituitary – Myotonic dystrophy • Drug-induced/toxic – Inherited – Schwarz-Jampel – Drug-induced11/19/2011 Jipmer physiologist 56
  57. 57. Diagnostic histological features of myopathies• Absence of neurogenic abnormalities• Necrotic muscle fibers• Basophilic (regenerating) myofibers• Fibrosis of the endomysium• Special pathological features (inflammatory cells, ragged red fibers etc.)11/19/2011 Jipmer physiologist 57
  58. 58. Muscle dystrophies• Hereditary myopathies, characterized by progressive weakness and muscle atrophy• Genetic defect of proteins constituting the sarcolemma-associated cytoskeleton system11/19/2011 Jipmer physiologist 58
  59. 59. Duchenne muscular dystrophy• First described in 1881- dystrophin gene discovered in the early 1980s11/19/2011 Jipmer physiologist 59
  60. 60. Duchenne muscular dystrophy• X-chromosome linked, recessive inheritance• 1 in 3500 live births,11/19/2011 Jipmer physiologist 60
  61. 61. Clinical features• Onset of weakness before age 5• Progressive weakness, proximal>distal, and muscle wasting• Gower’s sign• Hypertrophy of calves,• psuedohypertrophy of deltoid, gluteal• Skeletal deformities• Cardiomyopathy• wheel chair dependence by the age of 12, respiratory infection at 16-18 years. Fatal in the third decade11/19/2011 Jipmer physiologist 61
  62. 62. Electrophysiology• EMG changes – rate of muscle fiber destruction and extent of regeneration.• Fiber loss-Low amplitude short duration MUPs,• Fiber degeneration- polyphasic MUPs• Necrosis - fibrillations with low amplitude and short duration• Nerve conduction studies – generally normal11/19/2011 Jipmer physiologist 62
  63. 63. • Elevated CPK levels to 20 to 100 folds• Variation in size and shape of muscle fibers and small groups of necrotic and regenerating fibers- muscle biopsy.• Absence of dystrophin gene in biopsied muscles or genetic defect analysis in WBCs11/19/2011 Jipmer physiologist 63
  64. 64. Management• No specific treatment• Physiotherapy• Aerobic exercise• Low intensity anabolic steroids• Prednisone supplements• Orthoses (orthopaedic appliances used for support)• Orthopaedic surgery• Critical care11/19/2011 Jipmer physiologist 64
  65. 65. Beckers muscular dystrophy• Allelic defect in DMD gene.• 10 times less frequent than DMD• Better prognosis. Patient lives upto 40-50 years.• Mental retardation and heart failure can occur• Muscle biopsy – variable muscle fiber size with aberrant large fibers. Endomysial fibrosis and fatty infiltration• Patchy staining of DMD gene11/19/2011 Jipmer physiologist 65
  66. 66. Gene Clinical feature PathophysiologyFascioscapulohumeral MD - AD FSHMD1B ge Progressive muscular weakness and atrophy Dystrophic myopathy with inflammatory infiltrates involving the face, scapular, proximal arm and peroneal muscles myopathic face,Oculopharyngeal MD - AD PABP2 Ptosis and extra ocular Dystrophic myopathy incl muscle weakness rimmed vacuolesEmery – Dreifuss MD – X - linked EMD, LMNA Triad of early Mild myopathic changes. contracture, humero- Absent emerin by peroneal weakness and immunohistochemistry cardiac conduction defectsCongenital –MD AR Laminin Neonatal hypotonia , Variable fiber size and alpha 2 muscle weakness extensive endomysial fibrosisCongenital –MD – Fukuyama type AR Fukutin Neonatal hypotonia and Variable fiber size and MR extensive endomysial fibrosis. CNS malformationCongenital –MD Protein o Neonatal hypotonia and Variable fiber size andWalker – warnburg type mannosyl MR, ocular malformation extensive endomysial 11/19/2011 Jipmer physiologist transferase 66 fibrosis. CNS, ocular malformation
  67. 67. Gene Clinical feature PathophysiologyLimb-girdle dystrophiesSarcoglycanopathies AR α, β, γ, δ sarcoglyc ge Starts between 2 and 20 years Normal dystrophin immunostaining, Α, β, γ, δ sarcoglycans ans Clinically indistinguishable abnormal from duchenne-dystrophy immunostaining with No cardiac involvement sarcoglycans , Genetic defect analysisMyotonic dystrophy AD CTG repeat •Myotonia: hyperexcitability Muscle biopsy showing expansion in a of muscle membrane mild myopathic changes gene on chr. 19 inability of quick muscle and grouping of atrophic relaxation fast fibres •Progressive muscular weakness and wasting, most prominent in cranial and distal muscles •Cataracts, frontal balding, testicular atrophy •Cardiac abnormalities, mental retardationMyotonia congenita AD, AR Mucle cl gene Myotonia (hyperexcitability Autosomal of the muscle membrane): dominant form: muscle stiffness and Thomsen, abnormal muscle autosomal relaxation, warm-up recessive form: phenomenon 11/19/2011 Becker Hypertrophied muscles Jipmer physiologist 67
  68. 68. Inflammatory myopathiesPATHOPHYSIOLOGY• Polymyositis and inclusion body myositis (IBM) have autoaggressive CD8 lymphocytes that appear to attack myofibers and suggest an autoimmune role.• However,a major question exists about the etiology of IBM.• Dermatomyositis is thought to be caused by auto antibodies, possibly targeting an antigen of the endothelium. Fiber injury may be caused by ischemia.11/19/2011 Jipmer physiologist 68
  69. 69. Dermatomyositis Polymyositis Incusion body myositisSub acute progressive Sub acute progressive Slowly progressiveweakness weakness weakness,proximal>distal proximal>distal proximal and distal.Children and adults, women adults, women adults, mostly menCharacteristic rash andperiorbital heliotrope.Electromyogram myopathic potentials, myopathic potentials,myopathic spontaneous spontaneouspotentials, spontaneous activityElevated serum creatine Elevated serum creatine Mildly elevated serumkinase activity. kinase activity creatine kinase or normal.inflammatory myopathy inflammatory myopathy : inflammatory myopathyaffecting chiefly the endomysium affectingchiefly the perimysium with 11/19/2011 Jipmer physiologist chiefly the endomysium, but 69
  70. 70. Polymyositis Inclusion body myositis Dermatomyositis11/19/2011 Jipmer physiologist 70
  71. 71. Congenital myopathies• Group of muscle disorders• Early onset• Slowly progressive• Hereditary• Generalised or proximal weakness and wasting• Hypotonia• Contractures• Normally or mildly elevated CPK• Normal or myopathic EMG11/19/2011 Jipmer physiologist 71
  72. 72. Congenital Central core disease Nemaline Myotubularmyopathies myopathy myopathyInheritence AD AD , AR XL, AD, ARGene RYR- 1 gene AD –NEM1 -TMP3 XL – MTM1 AR- NEM2 - NEB AD – DNM2 AR- NEM3 - ACTA AR – BIN1 AR- NEM4 – TMP2 AR- NEM5 - TNNT1 AR- NEM7 - CFL2Clinical features Early onset hypotonia Chilhood weakness Severe congenital and weakness . .variable hypotonia. Floppy Floppy infant . presentation. infant .poor Associated skeletal Floppy infant prognosis deformitiesPathophysiology Cytoplasmic cores Aggregates of Abundance of are distinct from subsarcolemmal centrally located surrounding spindle shaped rods nuclei involving the 11/19/2011 Sarcoplasm. Jipmer physiologist majority of muscles 72
  73. 73. Distal myopathies• Welander distal myopathy• Miyoshi distal myopathy11/19/2011 Jipmer physiologist 73
  74. 74. Metabolic myopathies• Disorders of muscle energy metabolism• Disorders of lipid metabolism• Mitochontrial myopathies11/19/2011 Jipmer physiologist 74
  75. 75. Mitochontrial myopathies• Kearn – sayre syndrome• Myoclonic – epilepsy with ragged red fibers• Mitochontrial myopathy, lactic acidosis, stroke• Mitochontrial myopathies with recurrent myoglobunuria• Mitochontrial DNA depletion syndrome• Progressive external opthalmoplegia and ragged red fibers11/19/2011 Jipmer physiologist 75
  76. 76. Endocrine myopathies• Thyrotoxic myopathies• Cushing syndrome and steroid myopathy• Myopathy associated with oarathyroid disorders.11/19/2011 Jipmer physiologist 76
  77. 77. Toxic myopathies• Myotonic disorders• Necrotizing myopathies• Acute muscle necrosis• Mitochontrial myopathy• Hypokalemic myopathy• Inflammatory myopathy• Autophagic myopathy• Focal myopathy• Envenomation myopathy11/19/2011 Jipmer physiologist 77
  78. 78. Muscle channelopathiesNa Cl Cachannelopathies channelopathies channelopathiesHyperkalemic Myotonia Malignantperiodic paralysis congenita hyperthermia (Thomsen and Becker type)Paramyotonia Hypokalemiccongenita periodic paralysisPotassiumaggravatedmyotonia11/19/2011 Jipmer physiologist 78
  79. 79. Treatmento There is no single treatment for myopathy.o Treatment of the symptoms to specific cause- targeting treatments.o Drug therapyo Physical therapyo Bracing for support,o Surgeryo Massage11/19/2011 Jipmer physiologist 79
  80. 80. Care !….the best rehabilitation method11/19/2011 Jipmer physiologist 80
  81. 81. 11/19/2011 Jipmer physiologist 81

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