TOC  | Neurology

Guillain-Barré syndrome  
REF: Ferri: Ferri's Clinical Advisor: Instant Diagnosis and Treatment, 2003

DEFINITION

• Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyradiculopathy (AIDP) predominantly affecting motor function.

SYNONYMS

• Acute polyneuropathy
• Ascending paralysis
• GBS

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 B infections

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 hypertension)
• Respiratory insufficiency (caused by weakness of intercostal muscles)
• Facial paresis, difficulty swallowing (secondary to cranial nerve involvement)

ETIOLOGY

Infection with C. jejuni often precedes GBS and is associated with axonal degeneration, slow recovery, and severe residual disability.

DIAGNOSIS

DIFFERENTIAL DIAGNOSIS

• Neuropathy from heavy metals (lead, arsenic)
• Neuropathy from systemic diseases (uremia, diabetes, amyloidosis, lupus and other collagen-vascular disorders, porphyria)
• Poliomyelitis
• Botulism, diphtheria
• Hysterical paralysis
• Deficiency states (alcoholism, vitamin B12 , folic acid)
• Hereditary polyneuropathies

WORKUP

1. Rule out other causes of neuropathy (see above).
2. Lumbar puncture:
   • Typical findings include elevated CSF protein (especially IgG) and presence of few
   • mononuclear leukocytes.
   • Normal values may be seen at the beginning of illness.
   • If the diagnosis is strongly suspected, a repeat lumbar puncture is indicated.
3. Electromyography reveals slowed conduction velocities; prolonged motor, sensory, and F-wave latencies are also present.

LABORATORY TESTS

• CBC may reveal early leukocytosis with left shift.
• Vitamin B12 level, folate, and heavy metal screening are indicated only in selected cases.

TREATMENT

NONPHARMACOLOGIC THERAPY

• 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

1. 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.
2. 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.
3. 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.

CHRONIC Rx

1. Ventilatory support: may be necessary in 10% to 20% of patients
2. Aggressive nursing care to prevent decubiti, infections, fecal impactions, and pressure nervepalsies
3. Monitoring and treatment of autonomic dysfunction (bradyarrhythmias or tachyarrhythmias, orthostatic hypotension, systemic hypertension, altered sweating)
4. Treatment of back pain and dysesthesia with low-dose tricyclics
5. Stress ulcer prevention in patients receiving ventilator support

DISPOSITION

• 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.

04162003