Demyelinating Degenerating & Motor Neuron Disorders

• Multiple Sclerosis

  An autoimmune condition where the body attacks the myelin of brain & spinal cord resulting in demyelination of destruction of myelin

  • W/O myelin - it creates gaps in insulation
• MS Assessment

  Initial Episode: double vision, blurred vision

  • Pt may have nystagmus

  Pt can experience muscle weakness, impairment of pain, temp, touch senses

  • Ataxia, intention tremors, sexual dysfunction, depression, euphoria, fatigue, cognitive abnormalities
  • Lhermitte sign: electric sensation down spine/extremities when head is flexed forward
• MS Management

  Supportive

  Pt among - 20-50 yo
• Guillain-Barré syndrome

  A rare disease where the immune system attacks portions of nervous system - cause is unclear

  • Pt report minor respiratory or GI infection prior to weakness
  • Infectious agent attacks body - agent is similar to myelin structure so body attacks myelin thinking it is the agent
• Guillain-Barré Syndrome Assessment

  Starts as weakness & tingling sensations in legs

  • Weakness moves up legs & starts to affect thorax & arms - may lead to paralysis
  • This can take few hours
  • Pt are prone to severe swings in PR & BP
• Guillain-Barré Syndrome Management


  Prehospital includes protecting airway & ventilating

  Monitor ECG & VS

  Established IV - be prepared to administer fluids
• Parkinson Disease

  A neurologic condition where environmental & genetic factors can place pt at risk for damage to certain neurons

  • Substania nigra is responsible for dopamine (neurotransmitter needed among other things) production if damaged or overused disease can result
• Parkinson Disease Assessment

  Gradual onset of symptoms over mo. to yrs

  1. Tremor: rest tremors & postural tremors common
  2. Postural instability: pt have stiff posture where they are stoped over & alters their gait - stiffness makes pt unsteady when walking
  3. Rigidity: causes pt to move in fits & starts
  4. Bradykinesia: slowing down of routine motions
  5. classic type of gait - shuffles in a straight line with feet close together
  6. Dementia, difficulty swallowing (aphagia), fatigue, dystonia
  7. Foot & leg contractions - leg arched or arm flexed across chest & abdomen
• Parkinson Disease Management

  Pre-hospital management is supportive - reorient pt if needed

  If pt has trauma, manage it
• Amyotrophic Lateral Sclerosis (ALS)


  AKA Lou Gehrig disease that strikes voluntary motor neurons

  • Cause is unclear & condition is more common in middle-aged men of any race
• ALS Assessment


  Fatigue, general weakness of muscle groups, fasciculations (muscle twitching), & difficulty doing routine activities

  • Pt may have difficult speaking - as condition progresses a loss of ability to walk, move arms, eat
  • Pt last 3-5yrs
  • Pt die from respiratory infections or other immobility complications
• ALS Management


  Monitor airway

  Pt may be on several devices such as ventilators or feeding pumps - IV ports - ask pt family abt them
• Bell Palsy


  Paralysis of facial muscles due to inflammation of the facial nerve (cranial nerve 7)

  • Episode is sudden & can easily be confused w/ stroke - one sided
  • S&S: eyelid ptosis, facial droop or weakness, excessive salivation, loss of ability to taste - episode can last up to 2wks to 2mo
  • Management: corticosteroid to reduce inflammation in addition to analgesics
• Dystonia


  Severe, abnormal muscle spasms that cause bizarre contortions, repetitive motions or postures

  • Can be a sign of another condition or be a condition itself
  • Doesn't impact pt's LOC
  • Primary happen for unknown reason
• Dystonia Assessment


  Spasms are involuntary & are often painful
• Dystonia Management


  Prehospital care is focused on ruining out other conditions - seizures, strokes or reactions to psychiatric medications

  • if it's a reaction to an antipsychotic medication then Benadryl 25mg (diphenhydramine) - ineffective in primary dystonia
  • Pain management may be appropriate
• Encephalitis

  Inflammation of brain

  Presentation: fever, HA, N/V, & general malaise

  • As condition progresses - changes in LOC, include behavioral & personality changes, stiff neck, photophobia, confusion, lethargy
• Meningitis


  Inflammation of the meninges - the outer covering of the CNS

  • Bacterial: upper respiratory infection, as it progresses pt has HA, still neck, fever, chills, seizures, inc. ICP
  • Infants: bulging fontanelles. high-pitched cry
  • Viral: similar to bacterial except inc. ICP is not seen
• Encephalitis & Meningitis Management


  Prehospital care is supportive

  • Meningitis: place a mask over mouths
• Abscesses

  Caused by infectious agent within the brain or spinal cord - destroys tissue.

  • Immune system tries to kill pathogen - if not body created a wall to prevent pathogen from spreading
• Abscesses Assessment & Management

  • Look for a low- or high-grade fever, persistent HA, drowsiness, confusion, generalized or focal seizures, N/V, hemiparesis, stiff neck
  • Manage S&S - pay attention to inc. ICP, seizures
  • Manage temp
• Poliomyelitis & Postpolio


  A viral infection transmitted by fecal oral route - vaccine developed, so pt with it have no been immunized

  • S&S: happen after 1wk of exposure - sore throat, N/V, stiff neck, diarrhea, muscle weakness, or paralysis
  • Management: Hydration, ventilation, airway support
• Peripheral Neuropathy


  A group of conditions where nerves leaving spinal cord damaged. Signals moving to or from the brain become distorted

  • Causes: trauma, toxins, tumors, metabolic disorders. autoimmune attacks
  • Assessment: loss of sensation, numbness, burning, pain, muscle weakness, paresthesia (sensation of tingling, skin-crawling)
  • Management: Supportive