Guillain-Barre Syndrome

Question 1

Guillain-Barre Syndrome

Answer 1

An Immune-Mediated Neuropathy
Acute and usually severe polyneuropathy
Incidence: about 1-4 cases per 100,000/yr
Males affected slightly more than females

Question 2

Clinical Presentation of GBS

Answer 2

Rapid, areflexic, ascending motor paralysis
Fluctuating BP
Postural hypotension
Cardiac dysrhythmias
Pain

Question 3

Subtypes of GBS

Answer 3

AIDP (Acute Inflammatory Demyelinating Polyneuropathy) is the most common type
Acute motor axonal neuropathy
M. Fisher syndrome—GQ1B serum send out antibody marker

Question 4

Acute Motor Axonal Neuropathy

Answer 4

most common after campylobacter infection, fulminate onset with acute resp failure and prolonged convalescence—axons do not grow quickly—unlike pure myelin defects in classic gbs=AIDP (acute demyelinating polyneuropathy)
EMG (electromyography) diagnosis delayed weeks—axon transection is positive on emg 10-21 days later. (( Myelin defects (AIDP) are readily findable in appx 3-7 days. ?earlier))
Rare diagnosis of axonal form

Question 5

M Fisher Syndrome symptoms and tx

Answer 5

Ataxia
Areflexia
Ophthalmoplegia: essential feature- eye movement defective in all ROM, looks like eyes are stuck
Weakness but no much numbness, bowel/bladder not affected
Antibody positive GQ1B – serum marker
Symptoms slowly progressive days to a week
No known treatment
Favorable prognosis after months

Question 6

2 symptoms required for a diagnosis of GBS

Answer 6

1. progressive weakness

2. generalized hypo or areflexia

Question 7

7 supportive symptoms for a diagnosis of GBS

Answer 7

1. rapid symptom progression ceasing at 4 wks
2. relative symmetry of paresis
3. some sensory signs
4. cranial n involvement
5. autonomic dysfunction-late
6. CSF has elevated protein (if WBC up, think viral)
7. EMG supports diagnosis: variable degrees of conduction slowing and conduction blocks
   Can progress to require a Ventilator in <1 week 15%

Question 8

2 Lab findings for GBS

Answer 8

CSF: elevated protein without pleocytosis
Can be normal if sx <48hr

ElectroDx tests (EDx): changes lag behind clinical presentation
Helps differentiate different types of GBS
Initial changes to EDx occur within a week for AIDP

Question 9

5 reasons to think it is something other than GBS

Answer 9

Fever or constitutional symptoms present at onset
R/O sepsis
Prominent bladder dysfunction early in the course
Think spinal cord disease, Botulism
CSF has increase in WBCs
Think viral myelitis, 
Asymmetric weakness
Space occupying lesion in cord or brain
Well-demarcated sensory level
Space occupying lesion in cord

Question 10

2 Treatments for GBS

Answer 10

High dose intravenous immune globulin (IVIG)
Five daily infusions

Plasmapheresis

Question 11

GBS Prognosis

Answer 11

Good
Only about 20% have persistent disability
Chronic pain, weakness or numbness
Persistent decreased reflexes
Can relapse, not often
Can develop CIDP (chronic inflammatory demyelinating polyneuropathy) rare

Question 12

Plasmapheresis

Answer 12

Four to five times in one week
May reduce the need for mech vent and increase chances of full recovery at 1yr
May have significant improvement by the end of the treatment week or after several weeks