neuromuscular disorders

• Guillain-Barre


  Inflammatory destruction of the myelin sheath around peripheral motor nerves
• Guillain-Barre


  • Elevated CSF proteins are diagnostic
  • Runs its course over several weeks
  • Unknown cause but viruses may be responsible
  • Stumbling and unsteady gait
  • Starts in the feet and works its way up (ground to brain)
  • Can be treated with immunoglobulin (Ig) infusion
  • Can be treated with plasmapheresis
  • Can be treated with analgesics
• Myasthenia Gravis


  Autoimmune disorder against acetylcholine receptors
• Myasthenia Gravis


  • Ptosis (eyelid droop) & diplopia (blurred vision)
  • Choking while eating, problems with speech earlier in the progression
  • Head to toe (mind to ground)
  • Often improves with rest, periods of remission, will progress over the years
  • Immunosuppressive drugs
  • Plasmapheresis
  • Often related to cancer of the thymus = thymectomy
  • Neostigmine (brand name – Prostigmin), Mestinon
  • Diagnosed with "tensilon challenge"
• Muscular dystrophy


  Progressive degeneration of striated muscles
• Muscular dystrophy


  • Hereditary condition
  • Resp. failure is cause of death
  • Weakness
  • Poor cough
  • Secretion retention
  • Aspiration pneumonia risk
  • Swallowing problems
  • Bronchial hygiene
• Poliomyelitis


  Viral inflammation of gray matter of the spinal cord
• Poliomyelitis


  • Initial infection in varying severity may cause paralysis or death – initial may only last 3 days
  • Post polio may include varying degrees of muscle weakness that may progress over years
  • Poor ventilation & secretion management
• Amyotrophic lateral sclerosis (ALS)


  Degeneration of lower motor and upper motor neurons of the spinal cord, medulla, and cortex
• Amyotrophic lateral sclerosis (ALS)


  • Genetic defect
  • Weakness, flaccid, and spastic paralysis, muscle atrophy, fasciculations (twitching)
  • Advances over 3-4 years and eventually effects breathing – mind not effected
• Spinal muscular atrophy (SMA)


  Genetic motor neuron disease -> progressive degeneration of motor neurons in the spinal cord
• Spinal muscular atrophy (SMA)

  • Weakness & wasting of the voluntary muscles
  • Treatment is symptomatic and supportive and includes treating pneumonia, curvature of the spine and resp. infections, orthotic supports and rehab, bronchial hygiene and cough assist
• Cerebral palsy


  Caused by anoxia or traumatic event
• Cerebral palsy


  • Inability to control muscle movement some have MR or seizures
• Multiple sclerosis


  Autoimmune disease secondary to a virus
• Multiple sclerosis

  • Causes inflammation of CNS, T cells and macrophages degrade the myelin sheath of the nerves
  • Destruction causes slowed nerve signals
  • Ages 20-40
  • Blurred vision, muscle weakness, paresthesia
  • Tremors, spasticity
• Spinal cord injuries


  • C1-C3 injury: resp. muscle paralysis; phrenic nerve emerges here
  • C4- C8 injury: quadriplegic w/ some resp. function, may lose some abdominal muscle function
• Botulism


  Food born bacterium, blocks acetylcholine receptors from crossing the NM junction
• Diaphragm paralysis


  • Unilateral: Caused by a tumor or idiopathic, Asymptomatic, PFT: decreases volumes, CXR: elevated hemidiaphragm, Diagnosed by fluoroscopy while sniffing: paradoxical upward movement on inspiration on affected side
  • Bilateral: Rare, Accessory muscles take over, CXR: bilateral elevation of diaphragm, Paradoxical movement on inspiration bilaterally, PFT: lung volumes half of normal
• Ventilatory parameters used to track decline in resp. functions

  • MIP/NIF
  • VC = 10 ml/kg
  • Ve = 5 L/m