Abstract Ref Number = APCP1106
MYASTHENIC CRISIS PRESENTING AS RECCURENT RESPIRATORY FAILURE A CASE REPORT
Waode Sitti Asfiah Udu,Elisabeth Siti Herini,Desy Rusmawatiningtyas
Department of Child Health Faculty of medicine Public Health and Nursing Universitas Gadjah Mada Dr Sardjito hospital Yogyakarta Division of Pediatric Neurology, Department of Child Health, Faculty of Medicine Public Health and Nursing , Universitas Gadjah MadaDr Sardjito Hospital, Yogyakarta Division of Pediatric emergency and intensive care, Department of Child Health, Faculty of Medicine Public Health and Nursing , Universitas Gadjah MadaDr Sardjito Hospital, Yogyakarta
Background : Myasthenia gravis (MG) is a relatively rare acquired, autoimmune neuromuscular disease characterized by autoantibody attack of acetylcholine receptors at the motor end plate of striated muscles, which results in variable muscle weakness. The incidence ranges between 1.7 and 21.3 per million inhabitants worldwide. MG has variable pattern of muscle involvement, severity, and clinical course. Approximately 15% to 20% of patients with MG will experience a Myasthenic crisis (MC), which muscle weakness can be severe enough to result in respiratory failure.
Case Presentation Summary : We report the case of a 14-year-old girl with MC who presented with recurrent respiratory failure. She had cough, sputum, difficulty swallowing and symptoms of upper and lower extremity weakness. Seven month previously, she was diagnosed with Guillain-Barre syndrome (GBS) and had received plasmapheresis and mecobalamin. She still had third time episode of respiratory failure who need mechanical ventilator support within seven month. We considered other causes of respiratory failure such as neuromuscular disorders, GBS or MG or multiple sclerosis. Diagnosis of MG was made based on neurophysiological studies. The electroneuromyography showed a decrement in response to repetitive nerve stimulation in various muscles. Diagnosis of MC based on symptoms of respiratory failure and increased of acetylcholine antibody titers 500 times from normal levels. She received pyridostigmine bromide and methylprednisolone showed clinical improvement. She successfully weaning the ventilator and symptoms weakness of extremities improved. During follow up, she still get one episode of respiratory failure within a year with upper respiratory tract infection as precipitants at the previous hospital. Unfortunately, she passed away after post cardiac arrest in this episode.
Learning Points/Discussion : In case of reccurent respiratory failure with mechanical ventilator support, it is important to consider the possibility of a neuromuscular disorder. Delayed MG diagnosis may present with MC.With proper diagnosis, management and identified factors precipitant, can reduce life-threatening complications of MC.
Keywords: Myasthenia gravis Myasthenic crisis reccurent respiratory failure