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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.
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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.
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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.
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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
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What stays intact with GBS?
- Cognitive function
- Level of consciousness
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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.
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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?
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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
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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.
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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.
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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
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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
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What is the primary feature of Myasthenia gravis?
weakness of skeletal muscle: muscles most often involved are eyelids, chewing, swallowing, speaking, and breathing.
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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
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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)
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