406 E2 - ALS & GBS

Question 1

ALS definiton

Answer 1

a rare, progressive neuro disorder characterized by the loss of upper and lower motor neurons -> resulting in progressive muscle weakness
etiology is unknown

Question 2

ALS survival outcomes

Answer 2

death usually occurs around 3 years after dx
d/t respiratory failure

Question 3

ALS risk factors

Answer 3

age: 40-70
gender: male
genetics
smoking

Question 4

ALS pathogenesis

Answer 4

motor neurons in the brainstem, cerebral cortex & spinal cord gradually degenerate -> death of neurons results in axonal degeneration, demyelination & sclerosis (scarring) -> damage causes motor neuros to no longer send messages to the muscles so the muscles cannot function

Question 5

excitotoxicty hypothesis

Answer 5

excessive levels of glutamate initiate a cascade of event that lead to neuron death

Question 6

glutamate is what type of neuron transmitter

Answer 6

excitatory neurotransmitter

Question 7

based on the excitotoxicty hypothesis, what is a treatment to improve ALS survival rates

Answer 7

giving riluzole (an antiglutaminergic drug) to decrease levels of glutamate

Question 8

ALS clinical manifestations

Answer 8

-weakness of upper & lower extremities
-muscle wasting & spasticity
-dysarthria, dysphagia, drooling
-cognitive & behavioral changes
-constipation
-sleep problems
-breathing problems

Question 9

Riluzole classification

Answer 9

glutamate inhibitor

Question 10

Riluzole indication

Answer 10

ALS

Question 11

Riluzole MOA

Answer 11

glutamate antagonist -> reduces damage to motor neurons

Question 12

Riluzole SE

Answer 12

dizziness, GI upset, hepatotoxicity

Question 13

Riluzole nursing considerations

Answer 13

this drug does increase life expectancy (but only by about 6 months) and slows function of degeneration

Question 14

GBS definition

Answer 14

autoimmune disorder where the myeline sheath is damaged by autoantibodies usually triggered by a viral infection (GI or resp) + few cases caused by bacterial infection (campylobacter jejuni, food borne illness), post surgery and very rarely w/ flu vaccines

Question 15

GBS classic symptoms

Answer 15

-initially: weakness/tingling in lower extremities then ascends to the upper portion of the body
-can vary from tingling to paralysis to complete quadriplegia & respiratory insufficiency

Question 16

GBS symptom progression

Answer 16

ascending from lower extremities to upper and then recovery descends from upper extremities to lower

Question 17

what is the biggest concern of GBS

Answer 17

weakness of the respiratory muscles causing respiratory distress

Question 18

GBS time progression

Answer 18

symptoms can increase over a time of hours to weeks but some decline rapidly (intubated and on vent within 24 hrs)

Question 19

GBS recovery

Answer 19

can be slow and might need trach

Question 20

GBS clincial manifestations

Answer 20

-uncoordinated movements
-numbness & decreased sensation
-loss of bowel/bladder control
-blurred vision
-difficulty breathing, swallowing and chewing

Question 21

is there a cure for GBS

Answer 21

no

Question 22

goal of GBS

Answer 22

reduce severity and accelerate recovery

Question 23

GBS pharm

Answer 23

-steroid therapy
-high dose immunoglobulin therapy (shortens severity, duration, & length of stay)

Question 24

GBS prognosis

Answer 24

-95% survival rate
-75% complete recover
-25-30% have residual weakness after 3 years
-about 3% may suffer relapse of muscle weakness & tingling post initial attack