Guillain-Barre Syndrome
Guillain-Barre Syndrome
The overall treatment is the same for all GBS variants. Since the disease can be fatal, optimal care is provided in a hospital setting with intensive care facilities. Excellent multidisciplinary care involving supportive care as well as specific therapy is needed to manage the disease.
Immunotherapy has been shown to accelerate recovery in patients with GBS, particularly when initiated early after motor symptoms appear. However, it is unnecessary in mild cases where no motor symptoms are exhibited. There are two forms of immunotherapy indicated for specific therapy of GBS, plasma exchange and intravenous immunoglobulin G (IVIG).
Plasma exchange is a process that removes or dilutes the circulating immune factors implicated in the pathogenesis of GBS. This procedure has been shown to reduce the need for mechanical ventilation and hospitalization time by hastening recovery in nonambulant patients who seek treatment within 4 weeks of the onset of neuropathic symptoms. The maximal benefit therapy is seen if it is initiated within the first 2 weeks of onset. The usual regimen of plasma exchange is 5 times over 2 weeks, with a total exchange of about 5 plasma volumes. The disadvantages of plasma exchange include rare complications, such as sepsis. Furthermore, the use of fresh frozen plasma is associated with the risk of acquiring viral infections such as HIV.
IVIG, which is easier to administer than plasmapheresis, is associated with fewer complications and is more comfortable for the patient. It is recommended for patients who cannot ambulate without assistance within 2 or 4 weeks of neuropathic symptom onset. The recommended dose is 0.4 g/kg per body weight daily for 5 consecutive days. As an added advantage, the patient's CD8+ T-cell function is enhanced by an unknown function by the use of IVIG.
Patients on IVIG may develop self-limiting, influenzalike symptoms including fever, myalgia, headache, nausea, and vomiting. Other side effects include aseptic meningitis, neutropenia, and hypertension. Caution must be used when administering IVIG to patients with congestive heart failure and renal insufficiency, and its use is contraindicated in those patients with a previous history of anaphylaxis to IVIG. The risk of serious hepatitis C infection transmission has been reduced significantly, following changes in preparation and purification.
While oral corticosteroids and IV methylprednisolone were once believed to be useful in the treatment of GBS due to their immune-mediated inflammatory mechanism, they are no longer used because they do not seem to offer any benefit over immunotherapy. Furthermore, there seems to be no added advantage of adding corticosteroids to an IVIG regimen.
Treatment
The overall treatment is the same for all GBS variants. Since the disease can be fatal, optimal care is provided in a hospital setting with intensive care facilities. Excellent multidisciplinary care involving supportive care as well as specific therapy is needed to manage the disease.
Immunotherapy
Immunotherapy has been shown to accelerate recovery in patients with GBS, particularly when initiated early after motor symptoms appear. However, it is unnecessary in mild cases where no motor symptoms are exhibited. There are two forms of immunotherapy indicated for specific therapy of GBS, plasma exchange and intravenous immunoglobulin G (IVIG).
Plasma exchange is a process that removes or dilutes the circulating immune factors implicated in the pathogenesis of GBS. This procedure has been shown to reduce the need for mechanical ventilation and hospitalization time by hastening recovery in nonambulant patients who seek treatment within 4 weeks of the onset of neuropathic symptoms. The maximal benefit therapy is seen if it is initiated within the first 2 weeks of onset. The usual regimen of plasma exchange is 5 times over 2 weeks, with a total exchange of about 5 plasma volumes. The disadvantages of plasma exchange include rare complications, such as sepsis. Furthermore, the use of fresh frozen plasma is associated with the risk of acquiring viral infections such as HIV.
IVIG, which is easier to administer than plasmapheresis, is associated with fewer complications and is more comfortable for the patient. It is recommended for patients who cannot ambulate without assistance within 2 or 4 weeks of neuropathic symptom onset. The recommended dose is 0.4 g/kg per body weight daily for 5 consecutive days. As an added advantage, the patient's CD8+ T-cell function is enhanced by an unknown function by the use of IVIG.
Patients on IVIG may develop self-limiting, influenzalike symptoms including fever, myalgia, headache, nausea, and vomiting. Other side effects include aseptic meningitis, neutropenia, and hypertension. Caution must be used when administering IVIG to patients with congestive heart failure and renal insufficiency, and its use is contraindicated in those patients with a previous history of anaphylaxis to IVIG. The risk of serious hepatitis C infection transmission has been reduced significantly, following changes in preparation and purification.
Corticosteroids
While oral corticosteroids and IV methylprednisolone were once believed to be useful in the treatment of GBS due to their immune-mediated inflammatory mechanism, they are no longer used because they do not seem to offer any benefit over immunotherapy. Furthermore, there seems to be no added advantage of adding corticosteroids to an IVIG regimen.