Guillain-Barre Syndrome Flashcards
What is Guillain-Barre Syndrome?
idiopathic acute or subacute polyneuropathy
increasing evidence to suggest post infectious cause
- typically follows GI or res infection by 2-4 wks
i. e. Campylobacter jejuni
Epidemiology of GBS?
M > F
rare in children < 2y/o
MC cause of acute atraumatic generalized paralysis in all age groups
Pathophys of GBS?
infection invokes immune response
> ab production
> ab attach to peripheral nerve cells
> macrophages attach peripheral nerves
> acute polyneuropathy
What are the different GBS variants?
Mainly demyelination: acute inflammatory demyelinating polyneuropathy
Mainly axonal loss: acute motor axonal neuropathy,, acute motor and sensory axonal neuropathy
Miller fisher varient: miller fisher syndrome
What is the MC variant of GBS? presentation?
Acute Inflammatory Demyelinating Polyneuropathy (AIDP) 80-90%
- progressive symmetric weakness
- absent or depressed DTRs
- paresthesias in hands and feet
Describe Acute Motor Axonal Neuropathy (AMAN)
high prevalence in Japan & china
DTRs may be preserved
NO sensory involvement
Describe Acute Motor and Sensory Axonal Neuropathy (AMSAN)
AMAN + sensory involvement
more severe than AMAN
Describe Miller Fisher syndrome
Ophthalmoplegia w/ ataxia and areflexia
only 25% develop extremity weakness
What 2 GBS variants will you see absent or depressed DTRs?
AIDP- absent or depressed
miller fisher syndrome-areflexia
How does GBS effect CSF?
macrophages damage the peripheral nerve dural attachment > breaks down CSF barrier > allows transudation of proteins into the CSF
clinical manifestations of GBS?
progressive symmetric ascending muscle weakness and diminished DTRs
peaks within 4 wks
no visible atrophy
neuropathic pain
gait disturbance with an ataxic component out of proportion to the muscle weakness
+/- cranial n. involvement, respiratory failure, autonomic disturbances RARE
GBS should be considered in any child who….
has an acute gait disturbance
What is essential for dx of GBS? What does it show?
CSF!
elevated protein with normal WBC
Timing:
- usually normal in 1sts 2-3 days
- most will demonstrate at 1 week
- protein levels peak at 3-6wks
Other dx studies?
NCS, EMG: don’t need for dx of GBS but required for classification
When should you consider an alternative dx?
Fever, CSF leukocytosis, meningismus, papilledema, painless weakness and fatiguability, persistent asymmetry of sxs