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     TOC  | Neurology

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

DEFINITION

SYNONYMS

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

ETIOLOGY

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

DIAGNOSIS

DIFFERENTIAL DIAGNOSIS

WORKUP

  1. Rule out other causes of neuropathy (see above).
  2. Lumbar puncture:
  3. Electromyography reveals slowed conduction velocities; prolonged motor, sensory, and F-wave latencies are also present.

LABORATORY TESTS

TREATMENT

NONPHARMACOLOGIC THERAPY

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

Patient education information may be obtained from the Guillain-Barré Foundation International, Box 262, Wynnewood, PA 19096; phone: (610) 667-0131.

     

04162003