Guillain-Barré syndrome medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editors-In-Chief: Priyamvada Singh, MBBS [2]

Overview

Guillain-Barré syndrome (GBS) is an acute, autoimmune, polyradiculoneuropathy affecting the peripheral nervous system, usually triggered by an acute infectious process. With prompt treatment of plasmapheresis followed by immunoglobulins and supportive care, the majority of patients will regain full functional capacity. However, death may occur if severe pulmonary complications and dysautonomia are present.

Medical therapy

Intensive care management

  • When symptoms are severe, the patient will need to go to the hospital for breathing help, treatment, and physical therapy.
  • Supportive care with monitoring of all vital functions is the cornerstone of successful management in the acute patient.
  • Of greatest concern is respiratory failure due to paralysis of the diaphragm.
  • Early intubation should be considered in any patient with
    • A vital capacity (VC) <20 ml/kg,
    • A Negative Inspiratory Force (NIF) <-25 cmH2O
    • More than 30% decrease in either VC or NIF within 24 hours
    • Rapid progression of disease
    • Autonomic instability

Immunotherapy

    • Once the patient is stabilized, treatment of the underlying condition should be initiated as soon as possible.
    • Either high-dose intravenous immunoglobulins (IVIg) at 400mg/kg for 5 days or plasmapheresis can be administered, as they are equally effective and a combination of the two is not significantly better than either alone.
    • Therapy is no longer effective after 2 weeks after the first motor symptoms appear, so treatment should be instituted as soon as possible.
    • High-dose immunoglobulin therapy (IVIg) is used to reduce the severity and length of Guillain-Barré symptoms. In this case, the immunoglobulins are added to the blood in large quantity, blocking the antibodies that cause inflammation.
    • IVIg is usually used first because of its ease of administration and safety profile, with a total of five daily infusions for a total dose of 2 g/kg body weight (.4kg each day).
    • The use of intravenous immunoglobulins is not without risk, occasionally causing hepatitis, or in rare cases, renal failure if used for longer than five days.
    • If plasmapheresis is chosen, a dose of 40-50 mL/kg plasma exchange (PE) is administered four times over a week. A method called plasmapheresis is used to remove proteins, called antibodies, from the blood.
    • The process involves taking blood from the body, usually from the arm, pumping it into a machine that removes the antibodies, then sending it back into the body.
    • Glucocorticoids]] have NOT been found to be effective in GBS.

Other treatments for preventing complications

References

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