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Disorders of the Neuromuscular Junction

Disorders of the Neuromuscular Junction. Myasthenia gravis Radim Mazanec, MD, PhD. Definition. An autoimmune disease due to an antibody mediated attack directed against nicotinic AchR at neuromuscular junction 10% have thymoma 70% have thymic hyperplasia. nerv e. muscle.

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Disorders of the Neuromuscular Junction

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  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. nerve muscle Synaptic antigens VGCC MuSK AChR

  4. 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

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

  6. Diagnostic algorhitm • Electromyography • AChR antibodies • MR of mediastinum – thymomas, hyperplasia

  7. Low frequency stimulation - decrement

  8. Treatment • Thymectomy • Plasma exchange in myasthenic crisis (TPE) • Human immune globuline (IvIg) • Corticosteroids + cytostatics • AChE inhibitors (pyridostigmine, distigmine)

  9. Myasthenic crisis • Respiratory insuficiency – paralysis of respiratory muscles • Assisted ventilation required • Affect 15-20% myasthenic patients • Females : males = 2 : 1 • Average age : 55 years

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

  11. Complication of crisis • Ateletactic pneumonia (40%) • Hypotension • Cardio-respiratory arrest • Pneumothorax

  12. 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

  13. Outcome • Duration of intubation : 13 days • Duration of hospitalization : 35 days • Tracheostomy cca 40% • Mortality cca 4%

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