als + gbs

Question 1

ALS

Answer 1

amyatrophic lateral sclerosis
lou gehrig disease
rare progressive neurogenerative disorder characterized by loss of motor neurons
upper and lower motor neurons -> voluntary muscles
death ~3 yr after dx d/t resp fail
unknown etiology

Question 2

ALS: rf

Answer 2

40-70
M
genetics
smoking

Question 3

ALS: patho

Answer 3

decrease in large motor neurons in brainstem and spinal cord (+ cerebral cortex), they gradually degenerate
therefore electrical and chemical messages from brain cant reach muscles
death of motor neurons -> demyelination, axonal degeneration, sclerosis (scarring)
cant send message to muscle so muscles dont work

Question 4

ALS: possible cause

Answer 4

excitotox hypothesis:
excessive levels of glutamate (excitatory NT) initiate cascade of events that l/t neuron death
we see high levels of glutamate in CSF and antichol (riluzole) improves survival

Question 5

ALS: cm

Answer 5

vary greatly, depend on which neurons are affected, most have spinal form
weak UE: sometimes begin in legs, muscle wasting (atrophy), spasticity (spinal form), lose dexterity, difficulty walking, hand weak
dysarthria, dysphagia, drool -> chew, swallow, speak
cog and behavioral changes
c -> immobile, diet, muscle weak
sleep problems
breathing -> resp fail

Question 6

Riluzole

Answer 6

Glutamate inhibitor
Glutamate antagonist (block receptor sites), reduce damage to motor neurons
Only drug approved for ALS
Dizzy, GI upset, hepatotox
Increase LE by maybe 6mo

Question 7

GBS:

Answer 7

acute ascending polyneuritis
autoimmune, myelin sheath degen by autoantibodies -> covering of axons that carry nerve impulses
onet: days - wk following viral infection (resp, GI)

Question 8

GBS: possible etiologies

Answer 8

viral, bacterial (campylobacter jejuni - food), post sx
flu vax

Question 9

GBS: cm

Answer 9

weak/tingle in LE
ascend cm: weak goes from lower to upper
descend tm: strength returns from upper to lower
severity of s increase over hrs to weeks
potentially life threatening if resp muscles involved -> trach, mech vent
uncoordinated movements -> falls
numb and decrease sensation, weak and tingle
loss of bowel/bladder control
blurred vision -> can be early s, doesnt follow ascending pattern
difficulty breathing, swallowing, chewing

Question 10

GBS: tm

Answer 10

no known cure
goal: decrease severity, accelerate recovery
steroid and immunoglobulin used in acute phase, some have recurrence
steroid
high dose immunoglobulin therapy (IV): shorten severity and duration, decrease length of stay

Question 11

GBS: prognosis

Answer 11

95% survive
75% completely recover
25-35% have residual weakness after 3 years
~3% suffer relapse of muscle tingle and weakness many years past initial attack
not everyone gets back to baseline