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Disorders of the Neuromuscular Junction

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Disorders of the Neuromuscular Junction Myasthenia gravis Radim Mazanec, MD, PhD Definition An autoimmune disease due to an antibody mediated attack directed against ... – PowerPoint PPT presentation

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Title: Disorders of the Neuromuscular Junction


1
Disorders of the Neuromuscular Junction
  • Myasthenia gravis
  • Radim Mazanec, MD, PhD

2
Definition
  • An autoimmune disease due to an antibody mediated
    attack directed against nicotinic AchR at
    neuromuscular junction
  • 10 have thymoma
  • 70 have thymic hyperplasia

3
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4
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5
Synaptic antigens
VGCC
AChR
MuSK
6
Epidemiology
  • Incidence 1 20 000 in USA
  • Women slightly higher incidence 3 2
  • Majority of the MG are young women in the third
    decade and middle aged men in 5th and 6th decade
  • Children account for 11 of all patients with MG

7
Clinical Features
  • Fluctuated muscle weakness and excessive
    fatiguability
  • Diplopia and ptosis
  • Bulbar weakness swalloving, dysarthria
  • Generalized weakness of the trunk and extremities

8
Diagnostic algorhitm
  • Electromyography
  • AChR antibodies
  • MR of mediastinum thymomas, hyperplasia

9
Low frequency stimulation - decrement
10
Treatment
  • Thymectomy
  • Plasma exchange in myasthenic crisis (TPE)
  • Human immune globuline (IvIg)
  • Corticosteroids cytostatics
  • AChE inhibitors (pyridostigmine, distigmine)

11
Myasthenic crisis
  • Respiratory insuficiency paralysis of
    respiratory muscles
  • Assisted ventilation required
  • Affect 15-20 myasthenic patients
  • Females males 2 1
  • Average age 55 years

12
Clinical features
  • Respiratory tract infection, pneumonia ( 38)
  • Respiratory failure 99
  • Oropharyngeal or ocular weakness 86
  • Arms and legs weakness 76

13
Complication of crisis
  • Ateletactic pneumonia (40)
  • Hypotension
  • Cardio-respiratory arrest
  • Pneumothorax

14
Treatment
  • ICU is required for assisted ventilation
  • Cardiopulmonary monitoring
  • Plasmapheresis ( 5 sessions) or IvIg 2g/kg in
    five consecutive days
  • Antithrombotic treatment
  • Antibiotics
  • Respiratory rehabilitation

15
Outcome
  • Duration of intubation 13 days
  • Duration of hospitalization 35 days
  • Tracheostomy cca 40
  • Mortality cca 4
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