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DNS

Guillain Barre Syndrome

Most common acute neuropathy

	1-2 per 100,000 population / year
	actual incidence may be higher
	some mildly affected individuals may not seek medical attention.

Affect healthy individual

	Most patients are healthy, active prior to its onset.
	Usually a sporadic disorder, although epidemics do occur.

Affect any age

The average age of onset is around 40 years.

Rare

over 80 years of age

or less than 2 years of age.

Recent Reclassification into

	acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
	acute motor and sensory axonal neuropathy (AMSAN)
	acute motor axonal neuropathy (AMAN)
	and other

In Western country

	acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
	Is the most common type

Time course

	progresses over a period less than 4 weeks
	most patients reaches maximal deficit within 2 weeks of onset
	recovery occurs over the ensuing weeks to months
	About two-thirds of all GBS patients describe an infectious syndrome within the 4 weeks prior to the onset of neuropathic symptoms.
	Upper respiratory tract infection is self reported about 40% of the cases and diarrhea in 15-20%

Symptoms

	Symmetric, ascending weakness
	Involving proximal and distal muscles
	Paresthesias and muscle pain may be presenting complaints
	Sensory impairment is usually minimal

Signs

Deep Tendon reflexes are lost early

Cranial nerve, particularly the facial nerve, is relatively common.

Respiratory failure about 30%

may have pharyngeal dysfunction.

Autonomic nerve involvement

fluctuations of BP

cardiac arrhythmias

bladder dysfunction

Diagnostic Criteria for Guillain-Barr� syndrome* (After Asbury et al)

Features required for diagnosis

Progressive muscle weakness with areflexia

Features that are supportive of the diagnosis

	Symmetric progression less than four weeks
	autonomic features
	cranial nerve involvement
	absence of fever

CSF and electrodiagnostic findings

Elevated protein without cells (albumino-cytological dissociation) (but protein may be normal early)

Electrodiagnostic studies suggestive of demyelination

	prolonged distal latencies
	abnormalities in F-wave responses or mild slowing of conduction velocities (may be normal in early stage of disease)

Features that cast doubt or exclude the diagnosis

	Significant asymmetry
	Persistent bowel or bladder disturbance
	Hexacarbon abuse or history of lead intoxication or porphyria

Treatment options for GBS

Treatment

Dose

Notes

Human Immunoglobulin

0.4g / kg iv qd x 5 days (total dose 2g). Start with slow infusion rate, increase rate of infusion as tolerated

Side effects: headache, renal failure

Plasmapharesis

4 to 5 exchanges over 8-10 days, total 250 ml/kg

Need Shiley catheter

Intubation

if FVC < 15 ml/kg

Heparin

5000 u sq q12

DVT prophylaxis

Steroid

not used

no benefit when used alone

More important

	Good Nursing care
	PT, OT, ST

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