Neuropathies & myopathies - an overview
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The document discusses neuropathies and myopathies, detailing their definitions, symptoms, classifications, and underlying causes. Neuropathies result from nerve damage impacting motor, sensory, and autonomic functions, with various etiologies including hereditary, metabolic, and toxic factors. Myopathies are neuromuscular disorders characterized by muscle weakness due to muscular fiber dysfunction and include several subtypes and clinical features.
Neuropathies & myopathies - an overview
- 1. Neuropathies & Myopathies Dr. Dinesh T, 11/19/2011 Jipmer physiologist Junior resident 1
- 2. Neuropathies 11/19/2011 Jipmer physiologist 2
- 3. Definition Damage to nerves which may be caused either by diseases or trauma to the nerve or as a component of systemic illness 11/19/2011 Jipmer physiologist 3
- 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 symmetrically 11/19/2011 Jipmer physiologist 4
- 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 system 11/19/2011 Jipmer physiologist 5
- 6. Symptoms in neuropathy A wide array of symptoms can occur when nerves are damaged o Paresthesia o Sensitivity to touch, Positive Pins and needles Tingling Burning Negative Numbness Deadness As if wearing shocks and walk 11/19/2011 Jipmer physiologist 6
- 7. In chronic course symptoms worse, muscle wasting, paralysis, or gland dysfunction 11/19/2011 Jipmer physiologist 7
- 8. Neuropathy - Signs • Distal sensory loss • Distal weakness and atrophy • Decreased or absent reflexes – Ankle jerks lost first 11/19/2011 Jipmer physiologist 8
- 9. Various classifications 11/19/2011 Jipmer physiologist 9
- 10. Etiological Classification of neuropathies Hereditary 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 neuropathies Inflammatory neuropathies • Immune mediated o Guillain-Barré syndrome o Chronic inflammatory demyelinating polyradiculoneuropathy • Infectious – Leprosy – Diphtheria – Varicella – zoster 11/19/2011 Jipmer physiologist 10
- 11. Medications Causing Neuropathies o 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 Amitriptyline 11/19/2011 Jipmer physiologist 11
- 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 chronic 11/19/2011 Jipmer physiologist 12
- 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. • 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 multiplex 11/19/2011 Jipmer physiologist 14
- 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 diabetes 11/19/2011 Jipmer physiologist 15
- 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 - axonapathy 11/19/2011 Jipmer physiologist 16
- 17. Segmental demyelination Dysfucntion 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. 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 polyneuropathy 11/19/2011 Jipmer physiologist 18
- 19. Uniform demyelinating sensorimotor poly neuropathy • Hereditary motor sensory neuropathy- type I, III,IV • Leucodystrophies • Tangier disease • Cockayne syndrome • Congenital cerebrotendinous xanthomatosis • Congenital hypomyelinating neuropathies 11/19/2011 Jipmer physiologist 19
- 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 hypertrophy 11/19/2011 Jipmer physiologist 20
- 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. Uniform demyelinating sensorimotor poly neuropathy- Electrophysiological studies ( NCS ) show o Uniform slowing of NCV o Similar NCV slowing in adjacent nerves o Absence of conduction block and temporal dispersion o Prolongation of F response commensurate with NCV slowing 11/19/2011 Jipmer physiologist 22
- 23. Segmental demyelinating motor more than sensory neuropathy o Acute inflammatory demyelinating poly radiculo neuropathy AIDP o Chronic inflammatory demyelinating poly radiculo neuropathy CIDP o Multifocal motor neuropathy o Paraproteinemia o HIV neuropathy o Lyme disease o Diphtheria o Penicillamine 11/19/2011 Jipmer physiologist 23
- 24. AIDP o The prototype o Distal paresthesia with symmetric weakness o Distal areflexia o Variants are pure motor, pure sensory, autonomic, relapsing, and Miller fisher types o Cranial nerves esp facial n and bulbar may be involved o Respiratory muscles are severely involved in about 25 11/19/2011 Jipmer physiologist 24
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- 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 distribution 11/19/2011 Jipmer physiologist 26
- 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. Segmental demyelinating motor more than sensory neuropathy Nerve conduction studies o Slowing of motor and sensory conduction velocity o Prolongation of terminal latency o Conduction block o Dispersion and prolonged or absent F waves 11/19/2011 Jipmer physiologist 28
- 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 neuropathy 11/19/2011 Jipmer physiologist 29
- 30. • Distal symmetric weakness and wasting with minimal sensory loss 11/19/2011 Jipmer physiologist 30
- 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 muscles 11/19/2011 Jipmer physiologist 31
- 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. 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 – ulcer 11/19/2011 Jipmer physiologist 33
- 34. Diabetic neuropathy • Predominant pathology is axonal neuropathy. • In chronic cases segmental demyelination also seen Pathophysiology – • 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 membrane 11/19/2011 Jipmer physiologist 34
- 35. Sensory axonal polyneuropathy • Nerve conduction studies • Diminished or absent SNAP amplitude in the setting of normal motor nerve conduction velocity 11/19/2011 Jipmer physiologist 35
- 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 etc 11/19/2011 Jipmer physiologist 36
- 37. • Paresthesia and dyesthesia of feet and distal legs • Wasting is marked • Loss of ankle reflex • Pathophysiology – evidence of degeneration of distal portion of axons 11/19/2011 Jipmer physiologist 37
- 38. Axonal type of mixed sensorimotor neuropathy Nerve conduction studies • Reduced or absent SNAP • CMAP amplitude decreases and motor conduction velocity also decrease in later stage EMG • Fibrillations and positive sharp waves are prominent in distal muscles. • Temporal dispersion on proximal stimulation is not found as in demyelinating neuropathies 11/19/2011 Jipmer physiologist 38
- 39. Mixed axonal loss and demyelinating neuropathy • Diabetes • Uremia • Paraproteinemia 11/19/2011 Jipmer physiologist 39
- 40. • Paresthesia, dyesthesia or numbness • Reduced vibration and two point discrimination • Pathophysiology – segmental demyelination and remyelination along with axonal degeneration 11/19/2011 Jipmer physiologist 40
- 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 CMAP 11/19/2011 Jipmer physiologist 41
- 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 lesions 11/19/2011 Jipmer physiologist 42
- 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 biopsy 11/19/2011 Jipmer physiologist 43
- 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. 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. 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. 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/oxycodone 11/19/2011 Jipmer physiologist 47
- 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. 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. Myopathies 11/19/2011 Jipmer physiologist 50
- 51. Definition Neuromuscular disorders in which the primary symptom is muscle weakness due to dysfunction of muscle fiber 11/19/2011 Jipmer physiologist 51
- 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 – increasing 11/19/2011 Jipmer physiologist 52
- 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 depressed 11/19/2011 Jipmer physiologist 53
- 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 SENSATION 11/19/2011 Jipmer physiologist 54
- 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 diseases 11/19/2011 Jipmer physiologist 55
- 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-induced 11/19/2011 Jipmer physiologist 56
- 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. Muscle dystrophies • Hereditary myopathies, characterized by progressive weakness and muscle atrophy • Genetic defect of proteins constituting the sarcolemma-associated cytoskeleton system 11/19/2011 Jipmer physiologist 58
- 59. Duchenne muscular dystrophy • First described in 1881- dystrophin gene discovered in the early 1980's 11/19/2011 Jipmer physiologist 59
- 60. Duchenne muscular dystrophy • X-chromosome linked, recessive inheritance • 1 in 3500 live births, 11/19/2011 Jipmer physiologist 60
- 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 decade 11/19/2011 Jipmer physiologist 61
- 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 normal 11/19/2011 Jipmer physiologist 62
- 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 WBCs 11/19/2011 Jipmer physiologist 63
- 64. Management • No specific treatment • Physiotherapy • Aerobic exercise • Low intensity anabolic steroids • Prednisone supplements • Orthoses (orthopaedic appliances used for support) • Orthopaedic surgery • Critical care 11/19/2011 Jipmer physiologist 64
- 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 gene 11/19/2011 Jipmer physiologist 65
- 66. Gene Clinical feature Pathophysiology Fascioscapulohumeral 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 vacuoles Emery – Dreifuss MD – X - linked EMD, LMNA Triad of early Mild myopathic changes. contracture, humero- Absent emerin by peroneal weakness and immunohistochemistry cardiac conduction defects Congenital –MD AR Laminin Neonatal hypotonia , Variable fiber size and alpha 2 muscle weakness extensive endomysial fibrosis Congenital –MD – Fukuyama type AR Fukutin Neonatal hypotonia and Variable fiber size and MR extensive endomysial fibrosis. CNS malformation Congenital –MD Protein o Neonatal hypotonia and Variable fiber size and Walker – warnburg type mannosyl MR, ocular malformation extensive endomysial 11/19/2011 Jipmer physiologist transferase 66 fibrosis. CNS, ocular malformation
- 67. Gene Clinical feature Pathophysiology Limb-girdle dystrophies Sarcoglycanopathies 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 analysis Myotonic 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 retardation Myotonia 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. Inflammatory myopathies PATHOPHYSIOLOGY • 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. Dermatomyositis Polymyositis Incusion body myositis Sub acute progressive Sub acute progressive Slowly progressive weakness weakness weakness, proximal>distal proximal>distal proximal and distal. Children and adults, women adults, women adults, mostly men Characteristic rash and periorbital heliotrope. Electromyogram myopathic potentials, myopathic potentials, myopathic spontaneous spontaneous potentials, spontaneous activity Elevated serum creatine Elevated serum creatine Mildly elevated serum kinase activity. kinase activity creatine kinase or normal. inflammatory myopathy inflammatory myopathy : inflammatory myopathy affecting chiefly the endomysium affecting chiefly the perimysium with 11/19/2011 Jipmer physiologist chiefly the endomysium, but 69
- 70. Polymyositis Inclusion body myositis Dermatomyositis 11/19/2011 Jipmer physiologist 70
- 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 EMG 11/19/2011 Jipmer physiologist 71
- 72. Congenital Central core disease Nemaline Myotubular myopathies myopathy myopathy Inheritence AD AD , AR XL, AD, AR Gene 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 - CFL2 Clinical features Early onset hypotonia Chilhood weakness Severe congenital and weakness . .variable hypotonia. Floppy Floppy infant . presentation. infant .poor Associated skeletal Floppy infant prognosis deformities Pathophysiology 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. Distal myopathies • Welander distal myopathy • Miyoshi distal myopathy 11/19/2011 Jipmer physiologist 73
- 74. Metabolic myopathies • Disorders of muscle energy metabolism • Disorders of lipid metabolism • Mitochontrial myopathies 11/19/2011 Jipmer physiologist 74
- 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 fibers 11/19/2011 Jipmer physiologist 75
- 76. Endocrine myopathies • Thyrotoxic myopathies • Cushing syndrome and steroid myopathy • Myopathy associated with oarathyroid disorders. 11/19/2011 Jipmer physiologist 76
- 77. Toxic myopathies • Myotonic disorders • Necrotizing myopathies • Acute muscle necrosis • Mitochontrial myopathy • Hypokalemic myopathy • Inflammatory myopathy • Autophagic myopathy • Focal myopathy • Envenomation myopathy 11/19/2011 Jipmer physiologist 77
- 78. Muscle channelopathies Na Cl Ca channelopathies channelopathies channelopathies Hyperkalemic Myotonia Malignant periodic paralysis congenita hyperthermia (Thomsen and Becker type) Paramyotonia Hypokalemic congenita periodic paralysis Potassium aggravated myotonia 11/19/2011 Jipmer physiologist 78
- 79. Treatment o There is no single treatment for myopathy. o Treatment of the symptoms to specific cause- targeting treatments. o Drug therapy o Physical therapy o Bracing for support, o Surgery o Massage 11/19/2011 Jipmer physiologist 79
- 80. Care !….the best rehabilitation method 11/19/2011 Jipmer physiologist 80
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