REF: Ferri: Ferri's Clinical Advisor: Instant Diagnosis and Treatment, 2003
Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating
polyradiculopathy (AIDP) predominantly affecting motor function.
INCIDENCE/PREVALENCE: 1 to 2 cases/100,000 persons annually; incidence
increases with age (0.8 cases/100,000 persons at age 18, 3.2 cases/100,000
persons at age 60)
RISK FACTORS: HIV, Campylobacter jejuni enteritis, upper respiratory
infections, Epstein-Barr viral infection, CMV infection, immunizations,
pregnancy, Hodgkin’s lymphoma, mycoplasma infections, hepatitis
PHYSICAL FINDINGS & CLINICAL PRESENTATION
Symmetric weakness, initially involving proximal muscles, subsequently involving
both proximal and distal muscles; difficulty in ambulating, getting up from
a chair, or climbing stairs
Depressed or absent reflexes bilaterally
Initial manifestations involving the cranial musculature or the upper extremities
(e.g., tingling of the hands) in some patients
Minimal to moderate glove and stocking anesthesia
Ataxia and pain in a segmental distribution in some patients (caused by
involvement of posterior nerve roots)
Autonomic abnormalities (bradycardia or tachycardia, hypotension, or
Respiratory insufficiency (caused by weakness of intercostal muscles)
Facial paresis, difficulty swallowing (secondary to cranial nerve involvement)
Infection with C. jejuni often precedes GBS and is associated with axonal
degeneration, slow recovery, and severe residual disability.
Neuropathy from heavy metals (lead, arsenic)
Neuropathy from systemic diseases (uremia, diabetes, amyloidosis, lupus and
other collagen-vascular disorders, porphyria)
Deficiency states (alcoholism, vitamin B12 , folic acid)
Rule out other causes of neuropathy (see above).
Typical findings include elevated CSF protein (especially IgG) and presence
Normal values may be seen at the beginning of illness.
If the diagnosis is strongly suspected, a repeat lumbar puncture is indicated.
Electromyography reveals slowed conduction velocities; prolonged motor, sensory,
and F-wave latencies are also present.
CBC may reveal early leukocytosis with left shift.
Vitamin B12 level, folate, and heavy metal screening are indicated only in
Close monitoring of respiratory function (frequent measurements of vital
capacity and pulmonary toilet),
because respiratory failure is the major complication in GBS
Frequent repositioning of patient to minimize formation of pressure sores
Prevention of thromboembolism with antithrombotic stockings and SC heparin
(5000 U q12h)
Emotional support and social counseling
ACUTE GENERAL Rx
Infusion of IV immunoglobulins (0.4 mg/kg/day for 5 days) has replaced
plasmapheresis as therapy of choice at many centers. The addition of
glucocorticoids (IV methylprednisolone) to IV immunoglobulins is controversial.
It may result in additional improvement, but it is often associated with
a high frequency of complications.
Early therapeutic plasma exchange (TPE, plasmapheresis), started within 7
days of onset of symptoms, is beneficial in preventing paralytic com-plications
in patients with rapidly progressive disease. It is contraindicated
in patients with cardiovascular disease (recent MI, unstable angina), active
sepsis, and autonomic dysfunction.
Mechanical ventilation may be needed if FEV is <12 to 15 ml/kg, vital
capacity is rapidly decreasing or is <1000 ml and PaO2 is <70, or the
patient is having significant difficulty clearing secretions and is aspirating.
Ventilatory support: may be necessary in 10% to 20% of patients
Aggressive nursing care to prevent decubiti, infections, fecal impactions,
and pressure nervepalsies
Monitoring and treatment of autonomic dysfunction (bradyarrhythmias or
tachyarrhythmias, orthostatic hypotension, systemic hypertension, altered
Treatment of back pain and dysesthesia with low-dose tricyclics
Stress ulcer prevention in patients receiving ventilator support
Mortality is approximately 3%.
Prognosis for full recovery is very good. Only 15% to 20% of patients may
have minor residual motor deficits.
Patient education information may be obtained from the Guillain-Barré
Foundation International, Box 262, Wynnewood, PA 19096; phone: (610) 667-0131.