Guillain-Bare Syndrome Flashcards

1
Q

what is guillain bare syndrome

A

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

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2
Q

which variant of GBS is most common

A

acute demyelinating inflammatory polyneuropathy (AIDP)

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3
Q

what is GBS characterized by

A

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

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4
Q

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

A

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

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5
Q

what triggers GBS?

A

can be idiopathic but half of cases occur after microbial infection

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

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6
Q

what infections cause GBS

A

bacterial - campylobacteriosis (undercooked food like poultry)

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

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7
Q

what does the progression of GBS look like?

A

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

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8
Q

how do you typically recover with GBS

A

proximal to distal

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9
Q

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

A

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

hyporeflexia or areflexia

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10
Q

do sensory impairments occur with GBS?

A

symptoms of paraesthesia and hyperesthesias onset way later than motor

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11
Q

which CN are involved?

A

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

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12
Q

what other symptoms are present with GBS?

A

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

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13
Q

how is GBS diagnoses

A

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

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14
Q

what are the required features for GBS by the NINDS?

A
  • 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
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15
Q

what are the supportive features for GBS by the NINDS?

A
  • 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
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16
Q

what features make GBS doubtful?

A
  • sensory > motor loss
  • marked, persistent asymmetry of weakness
  • bowel/bladder dysfunction
  • severe pulmonary dysfunction with little or no limb weakness
  • fever at onset
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17
Q

what is AIDP

A

acute inflammatory demyelinating polyradiculoneuropathy

  • progressive, symmetrical muscle weakness, absent or depressed DTRs
  • precedes illness
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18
Q

miller fisher syndrome

A
  • opthalmoplegia, ataxia, areflexia
  • 25% develop extremity weakness
19
Q

what is AMAN

A

acute motor axonal neuropathy

  • axonal involvement
  • muscle weakness with occasional preservation of DTRs, sensory spared
20
Q

what is AMSAN

A

acute motor-sensory axonal neuropathy

  • axonal involvement
  • motor and sensory nerves impacted
21
Q

what are the 4 variants of GBS

A

AIDP
miller-fisher syndrome
AMAN
AMSAN

22
Q

what is the main management goal

A

control inflammatory response bc no cure

23
Q

what are the two meds you can receive

A

intravenous immunoglobulin (IVIg)
plasma exchange (plasmapheresis)

24
Q

what IVIg

A

intravenous immunoglobulin

  • plasma products made of antibodies extracted from blood
25
Q

what does IVIg do?

A

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

26
Q

benefits of IVIg?

A

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

27
Q

what is plasma exchange?

A

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

28
Q

how long is plamapheresis treatment

A

5 exchanges over 2 wks

29
Q

when is plasmapheresis recommended

A

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

30
Q

benefits of plasmapheresis

A

reduced nerve damage and faster clinical improvement

31
Q

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

A

no - before or after

32
Q

what are some potential complications with GBS?

A

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

33
Q

generally what does prognosis look like with GBS?

A
  • 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
34
Q

what are some negative prognostic indicators?

A

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

35
Q

what is the EGRIS

A

risk of developing respiratory failure in first week of admission

36
Q

what does the EGRIS look at

A

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

37
Q

what is the EGOS

A

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

38
Q

at week 1, what outcome measure do you do

A

modified erasmus GBS outcome score mEGOS

39
Q

what does mEGOS look at

A

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

40
Q

at week 2, what outcome measure do you do

A

erasmus GBS outcome score EGOS

41
Q

what does the EGOS look at

A

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

42
Q

what are 2 specific outcome measures for GBS

A

GBS disability scale
overall disability sum score ODSS

43
Q

what is CIDP

A

chronic inflammatory demyelinating polyneuropathy