Neuropathy& myopathy

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  • 1. MYOPATHIES & NEUROPATHIES www.freelivedoctor.com

2. NEUROPATHIES, MYOPATHIES

  • NEUROPATHIES (7)
    • Inflammatory
    • Infectious
    • Hereditary (HMSN-I)
    • HMSN-II, HMSN-III
    • Acquired Toxic/Metabolic
    • Traumatic
    • Neoplasms
  • MYOPATHIES (9)
    • Denervation
    • Dystrophies
    • Ion Channel
    • Congenital
    • Genetic Metabolic
    • Inflammatory
    • Toxic
    • NeuroMuscular Junction
    • Neoplasms

www.freelivedoctor.com 3. www.freelivedoctor.com 4. www.freelivedoctor.com 5. www.freelivedoctor.com 6. www.freelivedoctor.com 7. www.freelivedoctor.com 8. www.freelivedoctor.com 9. www.freelivedoctor.com 10. www.freelivedoctor.com 11. www.freelivedoctor.com 12. www.freelivedoctor.com 13. www.freelivedoctor.com 14. www.freelivedoctor.com 15. www.freelivedoctor.com 16. www.freelivedoctor.com 17. www.freelivedoctor.com 18. www.freelivedoctor.com 19. www.freelivedoctor.com 20. www.freelivedoctor.com 21. www.freelivedoctor.com 22. GENERAL Reactions

  • NERVE
    • DEMYELINATION (segmental)
    • AXONAL DEGENERATION
    • NERVE REGENERATION
    • REINNERVATION
  • MUSCLE FIBER
    • NECROSIS
    • VACUOLIZATION
    • REGENERATION
    • ATROPHY
    • HYPERTROPHY

www.freelivedoctor.com 23. www.freelivedoctor.com 24. www.freelivedoctor.com 25. www.freelivedoctor.com 26. www.freelivedoctor.com 27. HYPERTROPHY, ATROPHY www.freelivedoctor.com 28. NEUROPATHIES, MYOPATHIES

  • NEUROPATHIES (7)
    • Inflammatory
    • Infectious
    • Hereditary (HMSN-I)
    • HMSN-II, HMSN-III
    • Acquired Toxic/Metabolic
    • Traumatic
    • Neoplasms
  • MYOPATHIES (9)
    • Denervation
    • Dystrophies
    • Ion Channel
    • Congenital
    • Genetic Metabolic
    • Inflammatory
    • Toxic
    • NeuroMuscular Junction
    • Neoplasms

www.freelivedoctor.com 29. NEUROPATHY , Inflammatory

  • Guillain-Barr
    • Preceded by influenza-like illness
    • NO actual specific etiologic agent isolated, autoimmume disease to myelin gangliosides most likely
    • Inflammation of a peripheral nerve
    • DEMYELINATION
    • ASCENDING paralysis

www.freelivedoctor.com 30. Guillain-Barr , (AIDP),A cute I nflammatory D emyelinating P olyneuropathy www.freelivedoctor.com 31. NEUROPATHY , Infectious

  • Leprosy
  • Diphtheria
  • V/Z (Varicella-Zoster)

www.freelivedoctor.com 32. www.freelivedoctor.com 33. www.freelivedoctor.com 34. N E U R O T O X I N C. DIPHTHERIAE www.freelivedoctor.com 35. Z O S T E R POSTHERPETIC NEURALGIA ZOSTER in DRG www.freelivedoctor.com 36. NEUROPATHY , Hereditary (defective myelination) ( H ereditaryM otor andS ensoryN europathy)

  • HMSN-I (Charcot-Marie-Tooth)
  • HMSN-II (Like CMT of the neurons)
  • HMSN-III (Palpable Nerves) (aka, Dejerine-Sottas)

www.freelivedoctor.com 37. PES CAVUM(S),in CMT www.freelivedoctor.com 38. NEUROPATHY , Toxic/Metabolic

  • Symmetric, Asymmetric
  • Sensory, Sensorimotor
  • Somatic, Autonomic
  • Focal, Multifocal

www.freelivedoctor.com 39. NEUROPATHY , Toxic/Metabolic

  • Diabetes Mellitus
  • Vitamin Deficiencies (many Bs, E)
  • Heavy Metals, Pb, As, etc.
  • Organic Compounds
  • CHEMO

www.freelivedoctor.com 40. DEMYELINATION www.freelivedoctor.com 41. NEUROPATHY , Traumatic

  • Laceration
  • Avulsion
  • Carpal Tunnel
  • Traumatic (amputation) Neuroma
  • Saturday Night Palsy
  • Morton Neuroma

www.freelivedoctor.com 42. TRAUMATIC NEUROMA Regenerating Axons and Glia (Schwann Cells), but with no direction www.freelivedoctor.com 43. MORTONS NEUROMA Traumatic Compression F>M Interdigital Intermetatarsal MEDIAL Plantar Nerve 3 rdCOMMON digital branch www.freelivedoctor.com 44. www.freelivedoctor.com 45. NEUROPATHY , Neoplastic

  • Benign: Schwannoma
  • Malignant: Malignant Schwannoma

www.freelivedoctor.com 46. www.freelivedoctor.com 47. AntoniA : PalisadedAntoniB : NON-Palisaded www.freelivedoctor.com 48. MYOPATHIES www.freelivedoctor.com 49. MYOPATHY , Denervation MUSCLE FIBERSCANNOTSURVIVE UNLESS THEY ARE INNERVATED PERIPHERAL NERVE PATHOLOGY ANTERIOR HORN CELL PATHOLOGY www.freelivedoctor.com 50. www.freelivedoctor.com 51. S PINALM USCULARA TROPHY

  • Childhood diseases
  • Chromosome #5 that harbors the survival motor neuron gene ( SMN1 )
  • Anterior Horn Cells
  • Often PAN-fascicular
  • More peripheral muscles

www.freelivedoctor.com 52. MYOPATHY , Dystrophic

  • Jerrys kids, no DYSTROPHIN
  • DUCHENNE (DMD), x-linked
  • BECKER (BMD) (less common, less severe, same chromosome)
  • Many others also, all of which have complex genetic patterns which have all been precisely defined
  • MYOTONIA is a common feature

www.freelivedoctor.com 53. NORMAL DMD www.freelivedoctor.com 54. www.freelivedoctor.com 55. www.freelivedoctor.com 56. www.freelivedoctor.com Limb Girdle Muscular Dystrophies Inheritance Locus Gene Clinicopathologic Features 1A Autosomal-dominant 5q31 Myotilin Onset in adult life with slow progression of limb weakness, but sparing of facial muscles; dysarthric speech 1B Autosomal-dominant 1q21 Lamin A/C Onset before the age of 20 years in lower limbs, progression during many years with cardiac involvement 1C Autosomal-dominant 3p25 Caveolin-3 (M-caveolin) Onset before the age of 20, clinically similar to type 1B 1D Autosomal-dominant 7p Unknown Limb girdle muscle weakness, adult onset 2A Autosomal-recessive 15q15.1-21.1 Calpain 3 Onset in late childhood to middle age; slow progression during 2030 years 2B Autosomal-recessive 2p13.3-q13.1 Dysferlin Mild clinical course with onset in early adulthood 2C Autosomal-recessive 13q12 -Sarcoglycan Severe weakness during childhood, rapid progression; dystrophic myopathy on muscle biopsy 2D Autosomal-recessive 17q21 -Sarcoglycan (adhalin) Severe weakness during childhood, rapid progression; dystrophic myopathy on muscle biopsy 2E Autosomal-recessive 4q12 -Sarcoglycan Onset in early childhood, with Duchenne-like clinical course 2F Autosomal-recessive 5q33 -Sarcoglycan Early onset and severe myopathy; dystrophic myopathy on muscle biopsy 2G Autosomal-recessive 17q11-q12 Telethonin Distal weakness with limb-girdle weakness in late childhood to adulthood; rimmed vacuoles in muscle cells 2H Autosomal-recessive 9q31-q34.1 Tripartite motif-containing protein 32 (TRIM32) Limb-girdle and facial weakness with onset in childhood, mild, slowly progressive course 57. MYOPATHY , Ion Channel Channelopathies

  • MYOTONIA/HYPOTONIC PARALYSIS
  • FAMILIAL, (genetic) DISEASES
  • TRIGGERED BY:
    • Exercise
    • Cold
    • Carb Intake
  • Classified by K+, K+, K+
  • MALIGNANT HYPERTHERMIA can be triggered off by anesthetic halogenated inhalation agents in some of these patients!!!

www.freelivedoctor.com 58. MYOPATHY , Congenital Floppy Babies

  • HYPOTONIC
  • FAMILIAL, (genetic) DISEASES
  • MANY TYPES, in most of which the precise genetic defects have been identified

www.freelivedoctor.com 59. MYOPATHY , Metabolic (genetic also)

  • LIPID
    • Mitoch. Enz. Def. LIPID ACCUMULATION
  • MITOCHONDRIAL
    • PARKING LOT mitochondria

www.freelivedoctor.com 60. www.freelivedoctor.com 61. PARKING LOT MITOCH. www.freelivedoctor.com 62. MYOPATHY , Inflammatory

  • DERMATOMYOSITIS
  • POLYMYOSITIS
  • INCLUSION BODY MYOSITIS
  • ALL HAVE UNCLEAR ETIOLOGIES

www.freelivedoctor.com 63. DERMATOMYOSITIS (often peri-vascular) www.freelivedoct