guillian barre syndrome

Question 1

what is guillian barre syndrome?

Answer 1

an acute demyelinating polyneuropathy

Question 2

how does the classic guillian barre patient present?

Answer 2

viral infection or diarrhoe prodrome (75%)

few weeks later becomes weak over days

first legs than arms

mild sensory changes
can be facial, bulbar and/or respiratory weakness

signs of lower motor neuron dysfunction

Question 3

what does lower motor neuron dysfunction indicate in guillian barre?

Answer 3

acute neuropathy

Question 4

what does the LP for guillian barre show?

Answer 4

high protein

Question 5

what does the NCS for guillian barre show?

Answer 5

demyelination

Question 6

what are the LMN lower motor neurone problem sings

Answer 6

decreased tone

distally weak (sometimes pyramidal)

absent reflexes

absent clonus

plantar responses flexor or absent

Question 7

what is chronic inflammatory demyelinating polyneuropathy?

Answer 7

chronic demyelinating neruopathy similar to guillain barre

Question 8

how does CIDP, chronic inflammatory demyelinating polyneuropathy typically present?

Answer 8

similar to GBS but slower
with relapses

not usually prodrome (ie diarrhea) OR resp failure

LMN signs

LP high protein, NCS demyelination

ICP headaches

facial weakness and opthalmoparesis

Question 9

how is CIDP treatable?

Answer 9

immunosurpressants

good evidence for steroids (lancet 2006)

steroid sparing agents may be useful

IViG

Question 10

in what ways is CIDP different to guillian barre?

Answer 10

doesnt usually occur with prodrome

is chronic rather than acute

autonomic dysfunction

GBS does not respond to steroids

Question 11

what point is nadir in CIDP and GBS?

Answer 11

guillian barre 8 weeks

Question 12

what tree conditions could be used to describe the continuum between GBS and CIDP

Answer 12

acute inflammatory demyelinating polyneuropathy (AIDP)

subacute inflammatory demyelinating polyneuropathy (SIDP)

chronic inflammatory demyelinating polyneuropathy (CIDP)

Question 13

how is guillian barre treatable?

Answer 13

with IViG

supportive care

NG feeding

steroids ineffective

Question 14

what is the incidence of guillian barre?

Answer 14

2 per 100,000

Question 15

specifically, how does GBS present?

Answer 15

ascending symmetrical weakness hours to days

pain 2/3

LMN signs +/- mild sensory signs

facial weakness

respiratory weakness
bulbar involvement
CVS instability
sphincter signs

Question 16

what are the GBS mimics?

Answer 16

acute spinal cord lesion

crtical illness polyneuropathy

CIDP

HIV, lyme (rare)

Question 17

what are the GBS chameleons?

Answer 17

AMAN
AMSAN
miller-fisher syndrome

Question 18

what tests should be performed for GBS and CIDP?

Answer 18

lumbar puncture

nerve conduction study (demyelination)

MRI (Exclude cord lesions)

infective serology

Question 19

in rare cases what antibody can be testes for in guillian barre?

Answer 19

ganglioside antibodies

Question 20

what happens to the F wave in GBS?

Answer 20

delayed

Question 21

what is prognosis for GBS?

Answer 21

most patients fully recover

residual disability

Question 22

what age does CIDP mostly affect?

Answer 22

any age but mostly 40-60

Question 23

what is the incidence of CIDP?

Answer 23

1:200,000

Question 24

what two types of CIDP are they?

Answer 24

relapsing and progressive

Question 25

what is the pathogenesis of GBS?

Answer 25

T cells mistake myelin as foreign

linterleukin2 involved

cytokines attact macrophages which attack myelin