GBS & ALS

  1. Define Guillain-Barre Syndrome (GBS)
    How is it characterized?
    • An acute, rapidly progressing, and potentially fatal form of polyneuritis. 
    • Characterized by ascending, symmetric paralysis that usually affects cranial nerves and the peripheral nervous system.
  2. What is the pathophysiology of GBS?
    How are cells affected by it?
    • When cellular and humoral immune mechanisms play a role in the immun reaction directed at the nerves.
    • The result is a LOSS of myelin, with edema and inflammation at the affected nerves (this is called demyelination). 
    • The muscle cells innervated by the damaged peripheral nerves undergo denervation (loss of nerve supply) and atrophy.
  3. What are clinical manifestations of GBS?
    Including: motor function, autonomic, most common symptom and most serious
    • Can range from mild to severe
    • Weakness of lower extremities (occurring over hours, days to weeks, and usually peaks at the 14th day) 
    • Paresthesia (numbness and tingling) is common, followed by paralysis. 
    • Hypotonia (reduced muscle tone) and areflexia (lack of reflexes) are common
    • Autonomic: 
    •  - orthostatic hypotension, as well as HTN
    •  - abnormal vagal responses (bradycardia, heart block, asystole) 
    •  - facial flushing and diaphoresis
    • Most common: pain is the most common (worse at night) which can include
    •  - Paresthesias: abnormal sensation, like tingling or prickling
    •  - muscular aches and cramps
    •  - hyperesthesias: excessive physical sensitivity, especially at the skin.
    • The most serious: respiratory failure, which occurs as paralysis progresses to the nerves that innervate the thoracic area.
  4. List triggering events of GBS
    • Viruses (1-3 weeks before)
    • In 30% of cases, GBS precedes Campylobacter jejuni gastroenteritis (illness usually caused by eating raw or undercooked poultry, contaminated water, or unpasteurized milk)
    • Zika or Swine flu
    • Can also be triggered by trauma or surgery
  5. What stays intact with GBS?
    • Cognitive function
    • Level of consciousness
  6. List diagnostics for GBS
    Which diagnostic is done to rule out MS?
    • Lumbar puncture: CSF which shows an elevated protein level (usually after 7-10 days) **normal protein is 0.15-0.45**
    • EMG and Nerve conduction studies 
    • A brain MRI done to rule out MS
  7. Identify the disability score number with GBS:
    0
    1
    2
    3
    4
    5
    6
    • 0: healthy
    • 1: minor sx and capable of running
    • 2: able to walk >10m w/out assistance
    • 3: able to walk 10m with help
    • 4: bedridden or chair bound
    • 5: requiring assisted ventilation
    • 6: dead
  8. What is the purpose of Plasmapheresis tx for GBS? 
    What is the timing of when it is used?
    What is another tx drug used for GBS?
    • It is used in the first 2 weeks of GBS, but beyond 3 weeks of dz onset, it has little value. 
    • It can remove plasma containing antibodies launching attack on the body.
    • Another tx: IVIG in high dose to block damaging antibodies
  9. List nursing considerations for GBS
    • Support and encourage pt. and their family
    • It is a slow process to recovery, taking months to years
    • 85-95% of pts will recover, but may have some residual weakness
    • Patients may have potential for aspiration, test for gag reflex and drooling
    • Assess respiratory fxn and skin if bedridden
  10. Define ALS
    How is it characterized
    What is the average life-span after dx?
    What is the usual onset?
    Is it more common with men or women?
    • Amyotrophic Lateral Sclerosis: a rare progressive neurologic disorder
    • It is characterized by loss of motor neurons, leading to death usually 2-6 years after diagnosis. 
    • Usual onset is usually between 40-70 years of age. 
    • More common in men, with a ratio of 2:1
  11. Describe the pathophysiology of ALS.
    What does late stage on a patient look like?
    • It is a neurodegenerative disorder, affecting both upper (brainstem) and lower (spinal cord), whose motor cells degenerate.
    •  - These dead motor neurons from brain are unable to produce or send signals for activation
    • **cognition not typically affected**
    • Late stage: respiratory paralysis and death
  12. What are typical symptoms of ASL?
    • Limb weakness
    • Dysarthia: difficult or unclear articulation of speech that is otherwise linguistically normal
    • Dysphagia
    • Muscle wasting as a result of denervation and lack of stimulation
  13. List nursing interventions for ALS (6)
    • 1. facilitate communication
    • 2. reduce risk for aspiration
    • 3. facilitate early identification of respiratory insufficiency
    • 4. decrease pain secondary to muscle weakness
    • 5. decrease risk of injury r/t falls
    • 6. provide diversional activities, such as reading and companionship
  14. List clinical manifestations of ALS
    • Muscle atrophy and weakness (increase in creatnine kinase labs)
    • Weak or poor gait
    • Muscle twitching or continuously contracted
    • Hyperreflexia of DTRs
    • Increased creatine kinase levels (normal = 0%)
    • Dysarthria (slurred speech)
    • dysphagia
    • Ineffective cough
    • Fluctuations in BP
    • Metabolic changes
  15. This drug for ALS, although it won't cure it, will slow its progression by reducing amount of glutamate in the brain. What is its name?
    Rilutek
  16. This new FDA approved drug in Aug. 2017, was shown to slow muscle decline by 33% in ALS patients. 
    What is its name and how is it taken?
    Edaravone: taken via IV for 14 days, then 14 days off
  17. Fill in: patients will ALS, u want to encourage daily fluid intake of __a__ mL/day, if swallowing allows.
    You want to encourage cough, deep breathe every __b__ hours, as well as use the incentive spirometer.
    • a. 2500
    • b. 2 hours
  18. How many weeks after a URI or GI infection, can s/s of GBS appear?
    S/s of gbs over hours, days, or weeks. When is the peaking window in days?
    • 1-3 weeks
    • 14 days peaking
  19. WFN requires the presence of these 3 things to diagnose ALS
    • 1. signs of lower motor neuron generation (LMN)
    • 2. signs of UMN
    • 3. Progressive spread of signs within a region or to other regions, PLUS with the abscence of:
    •  - electrophsiological evidence of other dz processes that might explain the signs of LMN and/or UMN degenerations
    •  - Neuroimaging evidence of other dz processes that might explain observed clinical signs
  20. While there is no specific diagnostic test for Parkinson's, a firm diagnosis can be made only when there is at least two of these three signs.

    Which one of is often the first sign?

    What do Lewy Bodies signify?
    • 1. tremor (often first sign)
    • 2. rigidity
    • 3. bradykinesia

    Lewy bodies: unusual clumps of protein found int he brains of patients with PD.
  21. Is parkinsonian tremor more prominent at rest or during activity? 

    How does stress affect it?
    • More prominent at rest
    • Stress and increased concentration can aggravate tremors.
  22. For diet consideration with PD, is it better to eat smaller, but more frequent meals, or larger, less frequent meals? Why?
    These two things can impair the absorption of Levodopa.
    • Smaller, but more frequent meals d/t being less exhausting than eating a large meal. 
    • Levodopa:can be impaired by protein and Vitamin B6
  23. Fill in:
    Myasthenia Gravis is characterized by an autoimmune process where the __a__ receptors are attacked.

    Because these sites are attached, it prevents molecules from attaching and stimulating ___b___
    • a. AChR 
    • b. muscle contraction
  24. What is the primary feature of Myasthenia gravis?
    weakness of skeletal muscle: muscles most often involved are eyelids, chewing, swallowing, speaking, and breathing.
  25. List diagnostics for MG
    • Pulmonary function test: see if breathing is affected
    • EMG
    • Tensilon test: reveals muscle contractility and aids in diagnosis of cholinergic crisis
    • Blood test: looking for anti-AChR antibodies
  26. List three drug types given for MG

    List other Tx
    What kind of surgical intervention might be done?
    • Anticholinesterase agents
    • Corticosteroids
    • Immunosppressive agents

    Plasmapheresis and IVIG

    surgical: Thymectomy or most commonly a DBS (deep brain stimulation)
Author
edeleon
ID
341804
Card Set
GBS & ALS
Description
ADN-D EXAM 1
Updated