NMD, GBS, MG, TETANUS

• Neuromuscular diseases

  Affect the lower motor neurons of the nervous system, which may include the muscles, nerves, or the neuromuscular junction
• Examples of neuromuscular diseases

  • Muscular dystrophies
  • Inherited neuropathies like Charcot-Marie-Tooth (CMT) disease
  • Duchenne's or Becker's Muscular Dystrophy (DMD)
  • Congenital myopathies
• Respiratory complications in pediatric neuromuscular diseases

  • Major cause of morbidity and mortality
• Weakness of the muscles of respiration results in

  1. Shallow breathing and ineffective cough

  2. Patients vulnerable to atelectasis, pneumonia

  3. Tracheal obstruction by retained secretions
• Assessment of the risk of respiratory complications includes

  1. Evaluating the patient's history

  2. Respiratory physical examination

  3. Measuring pulmonary function and gas exchange
• Treatment options


  • Methods of assisted cough
  • Quad coughing
  • Mechanical Insufflation - Exsufflation (MIE) device
  • Ventilatory muscle training – diaphragmatic breathing
  • Mucociliary clearance techniques: chest physiotherapy, use of positive expiratory pressure device
• Mucociliary clearance techniques


  • Chest physiotherapy (Percussion, vibration/shaking, postural drainage)
  • Use of positive expiratory pressure device (ex. Flutter valve)
• Assisted cough devices


  • Chest Wall Oscillators
  • MIE Device
  • Assisted (Quad) Cough
• Airway care management


  • Tracheostomy
  • Suctioning
  • Humidity and aerosol therapy
  • Mechanical ventilation (Invasive or NIV)
• Guillain-Barre Syndrome (GBS)


  • Affects peripheral nerves; ascending paralysis
  • Impairs motor function and sensation
• Incidence of GBS: 0.4 – 1.4 cases/100,000 children
• Cause of GBS


  History of acute illness with variety of infectious agents: Campylobacter jejuni, cytomegalovirus, Mycoplasma pneumoniae, Haemophilus influenzae, Epstein-Barr virus; recent immunization (rare)
• GBS Management and Treatment

  • Plasmapheresis
  • Intravenous immunoglonulin
  • Enteral (tube feeding) or Parenteral nutrition – if with dysphagia
  • Vital signs monitoring
  • Vital capacity monitoring; NIF or PEFR (bedside)
  • Mechanical ventilation – if VC deteriorates to <20 ml/kg
• Myasthenia Gravis (MG)


  • An autoimmune disorder blocking the proper function of acetylcholine resulting to a defect in nerve impulse transmission to muscles
  • Descending paralysis
• Types of MG


  • Congenital
  • Neonatal
  • Juvenile
• MG in children


  • Can lead to respiratory muscle weakness and fatigue; may be triggered by infection, fever and stress
  • Poor cry and suck, facial muscle weakness and ptosis (drooping of eyelids)
• MG Management and Treatment

  • VC, NIF or PEFR monitoring
  • Aggressive bronchial hygiene
  • Non-invasive ventilation when required
  • Anticholinesterase medication
  • Use of apnea monitors
  • CPR when needed
  • Thymectomy – if patient has thymoma
  • Plasmapheresis and IV immunoglobulin – if with exacerbations
• Tetanus


  • Caused by a neurotoxin released by a spore-forming bacteria, Clostridium tetani
  • Bacteria enters the body through deep wounds or damaged cutaneous tissues
• Tetanus symptoms


  • Facial muscle rigidity and spasms resulting to trismus (lockjaw)
  • Flat-lipped grimace caused by facial muscle tightness – risus sardonicus
  • Opisthotonic posturing with full body spasms and rigidity
  • Respiratory involvement: chest wall muscle and diaphragmatic rigidity and dysfunction, laryngeal/glottic spasms
  • Arrhythmias leading to cardiac arrest
• Tetanus prevention: childhood immunization
• Tetanus Management and Treatment

  • Mechanical ventilation
  • Tracheostomy – for long term ventilatory support
  • Cardiovascular support – fluids, antiarhythmics, vasopressors
  • Pain medication – benzodiazepines
  • Enteral tube placement
  • Tetanus immunoglobulin
  • Metronidazole – antibiotic