Guillain-Bare Syndrome Flashcards
what is guillain bare syndrome
acute, immune-mediated, inflammatory, demylinating disorder with potential for chronic implications - immune system attacks schwann cells in PNS
which variant of GBS is most common
acute demyelinating inflammatory polyneuropathy (AIDP)
what is GBS characterized by
rapidly progessive ascending motor weakness and diminished reflexes - sensory, autonomic & brainstem abnormalities may occur
incidence of GBS, who is at most risk and at what age?
peaks in young adults 5th-8th decade - increases with age >60yrs
males > females
what triggers GBS?
can be idiopathic but half of cases occur after microbial infection
also - autoimmune disease, allergic, HIV/herpes, vaccines, surgery, post-natal
what infections cause GBS
bacterial - campylobacteriosis (undercooked food like poultry)
viral - hx of URI or GI infection, zika, epstein-barr, COVID
what does the progression of GBS look like?
progression for 12-24 hours then a period of nadir for 2-4wks then recovery begins
how do you typically recover with GBS
proximal to distal
motor or sensory dominant disease? and what does it look like?
motor - rapidly progresses
symmetrical, distal to proximal
leg weakness then arm weakness
hyporeflexia or areflexia
do sensory impairments occur with GBS?
symptoms of paraesthesia and hyperesthesias onset way later than motor
which CN are involved?
CN VII (facial) - smile, frowning, whistling, drinking through straw
CN III, IV, VI - double vision
CN IX, X - dysphagia and laryngeal paralysis
what other symptoms are present with GBS?
a lot of pain - neuropathic and msk
autonomic dysfunction - tachy, arrhythmias, OH, wide flucutuations of BP
respiratory difficulties
how is GBS diagnoses
clinical exam
CSF exam - increased protein levels
nerve conduction tests - reduced amplitude, decreased conduction
MRI - swelling/thickening of spinal nerve roots
what are the required features for GBS by the NINDS?
- 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
what are the supportive features for GBS by the NINDS?
- 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
what features make GBS doubtful?
- sensory > motor loss
- marked, persistent asymmetry of weakness
- bowel/bladder dysfunction
- severe pulmonary dysfunction with little or no limb weakness
- fever at onset
what is AIDP
acute inflammatory demyelinating polyradiculoneuropathy
- progressive, symmetrical muscle weakness, absent or depressed DTRs
- precedes illness
miller fisher syndrome
- opthalmoplegia, ataxia, areflexia
- 25% develop extremity weakness
what is AMAN
acute motor axonal neuropathy
- axonal involvement
- muscle weakness with occasional preservation of DTRs, sensory spared
what is AMSAN
acute motor-sensory axonal neuropathy
- axonal involvement
- motor and sensory nerves impacted
what are the 4 variants of GBS
AIDP
miller-fisher syndrome
AMAN
AMSAN
what is the main management goal
control inflammatory response bc no cure
what are the two meds you can receive
intravenous immunoglobulin (IVIg)
plasma exchange (plasmapheresis)
what IVIg
intravenous immunoglobulin
- plasma products made of antibodies extracted from blood
what does IVIg do?
blocks macrophage and antibody binding
boosts antigen production
prevents further myelin loss and axonal loss
benefits of IVIg?
significant improvements of 50-75% of pt’s
aids in sustain remission
what is plasma exchange?
plasmapheresis
removes blood plasma then separating into blood and cells then injecting the cells back into the blood stream
how long is plamapheresis treatment
5 exchanges over 2 wks
when is plasmapheresis recommended
when pt’s are not able to walk 10m without assistance
benefits of plasmapheresis
reduced nerve damage and faster clinical improvement
can you treat pt’s during receival of either of the medications?
no - before or after
what are some potential complications with GBS?
respiratory impairment and failure - vented
autonomic instability
pain
pneumonia
prolonged hospitalizations and immobility
relapse if treatment is inadequate**
generally what does prognosis look like with GBS?
- 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
what are some negative prognostic indicators?
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
what is the EGRIS
risk of developing respiratory failure in first week of admission
what does the EGRIS look at
days btw onset of weakness and hospital admission
facial or bulbar weakness at time of admission
UE/LE strength at time of admission
what is the EGOS
prognostic scoring system can be used at 1 and 2 wk after admission to estimate ability to walk at 6 months
at week 1, what outcome measure do you do
modified erasmus GBS outcome score mEGOS
what does mEGOS look at
age at onset
preceding diarrhea in last 4 wks
UE/LE strength at day 7 admission
at week 2, what outcome measure do you do
erasmus GBS outcome score EGOS
what does the EGOS look at
age at onset
prededing diarrhea in last 4 wks
GBS disability score at 2wk after hospital admission
what are 2 specific outcome measures for GBS
GBS disability scale
overall disability sum score ODSS
what is CIDP
chronic inflammatory demyelinating polyneuropathy
