Neuromuscular Junction Disorders

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Rosie435
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127931
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Neuromuscular Junction Disorders
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2012-01-15 11:54:04
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Neuromuscular Junction Disorders
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Neuromuscular Junction Disorders
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  1. Myastheinia Gravis (MG) Symptoms
    • 1. Fatigue muscles on sustained/ repeated use
    • 2. Improves after rest
    • 3. Effects:
    • 1.Diplopia
    • 2. Ptosis
    • 3. Dysarthria
    • 4. Dysphagia
    • 5. Masticatory weakness
    • 6. Lower/ Upper extremety weakness
    • 7. Generalized weakness
  2. MG Investigations
    1. Tensiolon Test- Endrophonium (reversible Anticholinesterase) injected IC results in rapid temorary improvement of weakness

    2. Repetitive N stimulation Tests- decriment in evoked potential following repetitive motor N stimulation

    3. Single Fibre Electromyography (EMG)- detects electrical potential generated by muscles cells

    4. Mediastinal CT/MRI- Thymoma
  3. Rx of MG
    • 1. Acetylcholinesterase Inhibitor
    • 2. Corticosteroids
    • 3. Immunosupprasants
    • 4. Plasmapheresis
    • 5. IV IgG
    • 6. Thymectomy
  4. Acetylcholinesterase Inhibitor in MG
    • Admin: Pyridostigmine- adjust freq and dose according to pt response
    • Ineffective in Occular Myasthenia
    • Muscarinic SE: Cramp, diarrhoea, Cholinergic crisis (Rx w/ Atropine)
  5. Corticosteroids in MG
    Prednisone 1mg/kg/day till max improvement achieved then slow taper over 6-12 months
  6. Immunosupprasants in MG
    • 1. Axothiaprine- monitor WBC and LFT
    • 2. Cycclophosphamide
    • 3. Cycolsporin A
  7. Plasmapharesis in MG
    • Indic:
    • 1. Life threatining
    • 2. Mysathenia Crisis
    • 3. Prior to thymectomy

    • SE:
    • 1. Hypotension
    • 2. Transient Cardiac dysrthmia
    • 3. Difficult Vasc access
  8. Thymectomy in MG
    • Indication:
    • 1. <40 years
    • 2. Thymoma

    80% improve significantly but full benifit may take years to appear

    Less effective in occular myasthenia
  9. Mysasthenica Crisis
    Features, Aetiol, Rx
    • 1. Features: Paralysis of respiratory muscles
    • 2. Aetiol: Inadequate Rx of MG or Infection
    • 3. Rx:

    • 1. Maintian AW
    • 2. Elective intubation (if FVC<15ml/kg)
    • 3. Plasmapharesis
    • 4. IV corticosteroids
  10. Cholinergic Crisis
    Features, Aetiology
    • Features:
    • 1. Pallory, sweaty
    • 2. Nausea, Vomiting
    • 3. Salivation
    • 4. Colicky abdominal pain
    • 5. Miosis (constricted pupil)

    Aetiology : Xs Acytilcholinesterase inhibitor = V High ACh
  11. Cholinergic Crisis Rx
    • 1. Mainitain AW
    • 2. Assisted Ventilliation
    • 3. IV Atropine (competitive antagonist of ACh)
    • 4. Temporary withdrawl of Anticholinesterase
    • 5. Immunosupp
  12. Botulism
    Organism, Types of toxins, Aetiology
    Toxin : Clostridium botulinum

    Toxins: A, B, E

    • Aetiology:
    • 1. Contaminated canned food
    • 2. Infected wounds
  13. Botulism- Clinical Features
    • Asym:
    • 1. Dilated pupils
    • 2. Dry Mouth
    • 3. Postural Hypotension
    • 4. Ileus
    • 5. Urine retention

    • Symm:
    • 1. Ptosis
    • 2. Squint
    • 3. Bulbar palsy
    • 4. Resp Muscle weakness
    • 5. Diminished reflexes

    Paralysis starting with muscles of face, spread to limbs and resp sys
  14. Botulisim: Pathophy
    • Prevents ACh release at:
    • 1. NMJ
    • 2. Autonomic synapses
  15. Botulisim Rx
    • 1. Monitor Vital Function
    • 2. Trivalent Antitoxin ABE
    • 3. Guanidine- facilitates ACh release
    • 4. Supportive Care
  16. Lambert-Eaton Myasthenic Syndrome
    Pathophy
    • 1. Autoantibodies against voltage gated Ca Ch in Pre synaptic memb
    • 2.Imparied release of ACh
  17. Lambert-Eaton MG
    Associated, Features
    Associated with lung CA

    • Features-
    • 1. Prox weakness
    • 2. Dry mouth
    • 3. Hyporeflexia

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