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Published on: November 9, 2017
[Guillain-Barré syndrome: epidemiological, clinical and therapeutic insight]
1Service de Réanimation Médicale, Hôpital Raymond-Poincaré, 92380 Garches.
Insights
Guillain-Barré syndrome treatment guidelines recommend plasma exchange (PE) or intravenous immunoglobulin (IVIg) for severe cases. Corticosteroids are ineffective for this neurological disorder.
Area of Science:
- Neurology
- Immunology
Context:
- Guillain-Barré syndrome (GBS) is a rare autoimmune disorder affecting the peripheral nervous system.
- The incidence of GBS is approximately 1.5 per 100,000 individuals annually.
- Severe neurological sequelae affect 10% of patients one year post-onset, necessitating specialized hospital care.
Purpose:
- To outline current treatment recommendations for Guillain-Barré syndrome based on clinical trial evidence.
- To specify indications for plasma exchange (PE) and high-dose intravenous immunoglobulin (IVIg) in different GBS severity forms.
- To highlight the ineffectiveness of corticosteroids in GBS treatment.
Summary:
- Plasma exchange (PE) is the first proven treatment for GBS, with specific protocols based on patient mobility and ventilation status.
- Four PE sessions are recommended for intermediate or severe GBS forms.
- High-dose IVIg (0.4 g/kg/day for 5 days) demonstrates equivalent efficacy to PE in intermediate and severe GBS.
- Ongoing studies aim to optimize IVIg dosage and PE frequency for various GBS severity levels.
Impact:
- Established evidence-based guidelines for GBS management, improving patient outcomes.
- Identified ineffective treatments (corticosteroids), preventing their use and associated side effects.
- Provided a foundation for further research into optimal GBS therapies, including dosage and treatment duration.
Abstract:
The average annual incidence of Guillain-Barré syndrome is 1.5 per 100 000. Mortality was about 5% in a recent clinical trial. Ten percent of patients have severe neurological sequelae one year after onset. For these patients, general care is essential and should be provided in appropriate hospital units. Corticosteroids, administered orally or intravenously are ineffective. Plasma exchange (PE) was the first treatment to demonstrate efficacy in randomized clinical trials. Indications have been recently specified. Patients who can walk must be given two PEs and two additional PEs in case of aggravation. Four plasma exchanges are sufficient in patients unable to walk unaided (intermediate form) or who are mechanically ventilated (severe form). No further PE is required if the patient fails to improve. High-dose intravenous immunoglobulins (0.4 g /kg daily for 5 days) and PE have equivalent efficacy in intermediate and severe forms. The optimal dose of IVIg and the number of Pes in the different severity forms are being assessed in an ongoing study.
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