Guillain-Bare Syndrome

Question 1

what is guillain bare syndrome

Answer 1

acute, immune-mediated, inflammatory, demylinating disorder with potential for chronic implications - immune system attacks schwann cells in PNS

Question 2

which variant of GBS is most common

Answer 2

acute demyelinating inflammatory polyneuropathy (AIDP)

Question 3

what is GBS characterized by

Answer 3

rapidly progessive ascending motor weakness and diminished reflexes - sensory, autonomic & brainstem abnormalities may occur

Question 4

incidence of GBS, who is at most risk and at what age?

Answer 4

peaks in young adults 5th-8th decade - increases with age >60yrs
males > females

Question 5

what triggers GBS?

Answer 5

can be idiopathic but half of cases occur after microbial infection

also - autoimmune disease, allergic, HIV/herpes, vaccines, surgery, post-natal

Question 6

what infections cause GBS

Answer 6

bacterial - campylobacteriosis (undercooked food like poultry)

viral - hx of URI or GI infection, zika, epstein-barr, COVID

Question 7

what does the progression of GBS look like?

Answer 7

progression for 12-24 hours then a period of nadir for 2-4wks then recovery begins

Question 8

how do you typically recover with GBS

Answer 8

proximal to distal

Question 9

motor or sensory dominant disease? and what does it look like?

Answer 9

motor - rapidly progresses
symmetrical, distal to proximal
leg weakness then arm weakness

hyporeflexia or areflexia

Question 10

do sensory impairments occur with GBS?

Answer 10

symptoms of paraesthesia and hyperesthesias onset way later than motor

Question 11

which CN are involved?

Answer 11

CN VII (facial) - smile, frowning, whistling, drinking through straw
CN III, IV, VI - double vision
CN IX, X - dysphagia and laryngeal paralysis

Question 12

what other symptoms are present with GBS?

Answer 12

a lot of pain - neuropathic and msk
autonomic dysfunction - tachy, arrhythmias, OH, wide flucutuations of BP
respiratory difficulties

Question 13

how is GBS diagnoses

Answer 13

clinical exam
CSF exam - increased protein levels
nerve conduction tests - reduced amplitude, decreased conduction
MRI - swelling/thickening of spinal nerve roots

Question 14

what are the required features for GBS by the NINDS?

Answer 14

• progressive, symmetrical weakness of legs and arms (sometimes just legs initially) - ranging from weakness to total paralysis of everything
• areflexia or decreased reflexes in weak limbs

Question 15

what are the supportive features for GBS by the NINDS?

Answer 15

• progression of symptoms <4wks
• relative symmetry
• mild sensory impairments or signs
• CN involvement, bilateral
• recovery starting 2-4wk after progression halt
• autonomic dysfunction
• pain
• no fever
• elevated protein in CSF
• electrodiagnostic abnormalities consistent w GBS

Question 16

what features make GBS doubtful?

Answer 16

• sensory > motor loss
• marked, persistent asymmetry of weakness
• bowel/bladder dysfunction
• severe pulmonary dysfunction with little or no limb weakness
• fever at onset

Question 17

what is AIDP

Answer 17

acute inflammatory demyelinating polyradiculoneuropathy

• progressive, symmetrical muscle weakness, absent or depressed DTRs
• precedes illness

Question 18

miller fisher syndrome

Answer 18

• opthalmoplegia, ataxia, areflexia
• 25% develop extremity weakness

Question 19

what is AMAN

Answer 19

acute motor axonal neuropathy

• axonal involvement
• muscle weakness with occasional preservation of DTRs, sensory spared

Question 20

what is AMSAN

Answer 20

acute motor-sensory axonal neuropathy

• axonal involvement
• motor and sensory nerves impacted

Question 21

what are the 4 variants of GBS

Answer 21

AIDP
miller-fisher syndrome
AMAN
AMSAN

Question 22

what is the main management goal

Answer 22

control inflammatory response bc no cure

Question 23

what are the two meds you can receive

Answer 23

intravenous immunoglobulin (IVIg)
plasma exchange (plasmapheresis)

Question 24

what IVIg

Answer 24

intravenous immunoglobulin

• plasma products made of antibodies extracted from blood

Question 25

what does IVIg do?

Answer 25

blocks macrophage and antibody binding boosts antigen production prevents further myelin loss and axonal loss

Question 26

benefits of IVIg?

Answer 26

significant improvements of 50-75% of pt's aids in sustain remission

Question 27

what is plasma exchange?

Answer 27

plasmapheresis removes blood plasma then separating into blood and cells then injecting the cells back into the blood stream

Question 28

how long is plamapheresis treatment

Answer 28

5 exchanges over 2 wks

Question 29

when is plasmapheresis recommended

Answer 29

when pt's are not able to walk 10m without assistance

Question 30

benefits of plasmapheresis

Answer 30

reduced nerve damage and faster clinical improvement

Question 31

can you treat pt's during receival of either of the medications?

Answer 31

no - before or after

Question 32

what are some potential complications with GBS?

Answer 32

respiratory impairment and failure - vented autonomic instability pain pneumonia prolonged hospitalizations and immobility relapse if treatment is inadequate**

Question 33

generally what does prognosis look like with GBS?

Answer 33

- 80% recover ambulation within 6mo - half may experience minor neurological deficits - DISTAL muscle weakness may persist, pain, fatigue - long term morbidity and mortality is low - recovery up to 2 years - relapses can occur, unlikely

Question 34

what are some negative prognostic indicators?

Answer 34

older age >60 vent support rapid onset, <7 days prior to admission avg distal motor response amp reduction to <20% of normal hx of GI illness - diarrhea

Question 35

what is the EGRIS

Answer 35

risk of developing respiratory failure in first week of admission

Question 36

what does the EGRIS look at

Answer 36

days btw onset of weakness and hospital admission facial or bulbar weakness at time of admission UE/LE strength at time of admission

Question 37

what is the EGOS

Answer 37

prognostic scoring system can be used at 1 and 2 wk after admission to estimate ability to walk at 6 months

Question 38

at week 1, what outcome measure do you do

Answer 38

modified erasmus GBS outcome score mEGOS

Question 39

what does mEGOS look at

Answer 39

age at onset preceding diarrhea in last 4 wks UE/LE strength at day 7 admission

Question 40

at week 2, what outcome measure do you do

Answer 40

erasmus GBS outcome score EGOS

Question 41

what does the EGOS look at

Answer 41

age at onset prededing diarrhea in last 4 wks GBS disability score at 2wk after hospital admission

Question 42

what are 2 specific outcome measures for GBS

Answer 42

GBS disability scale overall disability sum score ODSS

Question 43

what is CIDP

Answer 43

chronic inflammatory demyelinating polyneuropathy