class 12 guillain-barre and ALS

Question 1

what is guillain-barre syndrome

Answer 1

an autoimmune disorder with acute attack of peripheral nerve myelin
-rapidly progressing, and potentially fatal form of polyneuritis
-symmetrical ascending paralysis with unknown cause

Question 2

pathophysiology of GBS

Answer 2

-cellular and humoral immune reaction directed at nerves
-segmental demyelination, edema, and nerve inflammation
-transmission of nerve impulses is stopped or slowed, muscle atrophy occurs
-considered a medical emergency d/t risk of resp distress

Question 3

acute stage of GBS

Answer 3

severe rapid weakness, decreased reflexes, numbness, pain, decreased vital capacity, facial muscle involvement
ANS involvement: bradycardia, sweating, hypotension, dysarythmias

Question 4

plateau stage of GBS

Answer 4

2-3 weeks after inital onset, symptoms cease to change
-autonomic function stabilizes

Question 5

recovery stage of GBS

Answer 5

symptoms improve, takes several months to 2 years to recover (usually 6m)
-muscle strength and function returns in reverse order, will need rehab

Question 6

diagnostic studies for GBS

Answer 6

-history & clinical signs
-spinal tap (isn’t diagnostic, rules out other conditions)
-electromyography (EMG) to assess for dec in muscle transmission
-rule out other conditions

Question 7

clinical manifestations of GBS

Answer 7

-mild to severe
-event occurs 2 weeks before s&s
-typically progress over hours to days
-symmetrical muscle weakness
-mild distal sensory symptoms of paresthesia
-absent or depressed DTR
-weakness usually begins in legs and progresses upwards (ascending)
-pain is a common symptom

Question 8

complications of GBS

Answer 8

-respiratory distress/failure
-cardiac dysfunction
-severe pain
-immobility
-tachycardia
-UTI
-fever
-immobility
-paralytic ileus
-muscle atrophy

Question 9

medical management of GBS

Answer 9

-requires intensive care management with continuous monitoring and respiratory support
-plasmapheresis and IVIG are used to reduce circulating antibodies
-prevent the complications of immobility (anticoags and TEDs)

Question 10

assessment for GBS

Answer 10

-important during acute phase->can go undetected early
-vitals
-ascending paralysis
-resp function
-ABG levels
-Gag, corneal, and swallowing refexes
-may need TPN or NG for short term

Question 11

nursing interventions for GBS

Answer 11

IV fluids/TPN/NG feeds
-ROM exercises, position changes, TEDs, SCDs
-establishing a form of communication
-frequent respiratory assessment
-diversional activities “we expect this to happen”

Question 12

goals of nursing management for GBS

Answer 12

-improve resp function
-improve mobility
-improve nutrition status
-effective communication
-decrease fear and anxiety
-absence of complications

Question 13

what is amyorophic lateral sclerosis (ALS)

Answer 13

“lou gehrig’s disease”
-rare progressive neurological disorder
-avg survival time is 3-5 years post dx

Question 14

pathophysiology of ALS

Answer 14

-d/t unknown reasons, motor neurons in the brain stem and spinal cord gradually degenerate
-dead motor neurons cannot produce or transport vital signals to muscles
-electrical or chemical messages originating in the brain do not reach muscles

Question 15

risk factors for ALS

Answer 15

-age
-family hx
-genetic aberrations now identified
-exposure to pesticides, chemicals, heavy metals (i.e. military service, athleticism, smoking, viruses)

Question 16

manifestations of ALS

Answer 16

-depends on location of affected neurons
-fatigue, progressive muscle weakness, cramps, fasciculations, and incoordination
-progessive weakness
-dysarthria & dysphagia
-atrophy of the muscles in the arms, trunk, legs
-anal and bladder spincter function remains intact but may have accidents

Question 17

early s&s of ALS

Answer 17

-tripping over nothing
-dropping things
-slurred or “thick” speech
-difficulty swallowing
-weight loss
-decreased muscle tone
-sob
-inc or dec reflexes
-uncontrollable laughing or crying
-feeling weak
-fatigue
-cramping/twiching
-stiffness or rigidity

Question 18

death of ALS is usually d/t

Answer 18

-infection
-respiratory failure
-aspiration
-has the option of MAID to avoid these

Question 19

signs of disease progression with ALS

Answer 19

-difficulty breathing
-coughing when eating or drinking
-difficulty forming works or projecting voice
-fatigue caused by muscle exhaustion
-reduced food intake and weight loss
-insomnia caused by discomfort
-excessive saliva or dry mouth

Question 20

diagnostic studies for ALS

Answer 20

-no definitive test
-detailed hx & s&s
-electromyography (EMG), nerve conduction study (NCS), MRI to rule out mimicking diseases

Question 21

how ALS is diagnosed

Answer 21

-clinical, electrophysical, or neuropathologic evidence of lower motor neuron degeneration
-evidence of upper motor neuron degeneration
-evidence of progressive spread of s&s within a region or to other regions
COMBINED with absence of evidence of other dx that explain’s patients neuron degenration and neuroimaging evidence of other dx that explains s&s

Question 22

medical management of ALS

Answer 22

no cure
-riluzole (rilutek) or edaravone (radicava): glutamate antagonist slows progression by dec the amount of glutamate in the brain
-nuedexta: used to tx emotional lability
-baclofen (lioresal), dantrolene sodium (dantrium), or diazepam (valium) may be useful for patients troubled by spasticity

Question 23

nursing interventions for ALS

Answer 23

-facilitating communication
-reduce aspiration risk
-early identification of resp insufficiency
-pain management
-dec risk of falls/injury
-support cognitive and emotional functions
-mod intensity, endurance exercises for the trunk and limbs to reduce spasticity