- acute monophasic paralyzing illness usually provoked by a preceding infection
- In the absence of disease-modifying treatment, most patients with GBS show continued progression for up to two weeks, followed by a plateau phase of about two weeks, and then recovery of function over several weeks to months
Supportive Care
- Supportive care is extremely important in Guillain-Barré syndrome (GBS) since up to 30 percent of patients develop neuromuscular respiratory failure requiring mechanical ventilation
- admitted to the ICU for close monitoring of respiratory, cardiac, and hemodynamic function
- Prophylaxis for deep vein thrombosis, bladder and bowel care, physical and occupational therapy, and psychological support are essential.
- Low molecular weight heparin and support stockings are recommended until patients are able to walk independently
Pain
- Neuropathic pain occurs in about 40 to 50 percent of patients
- Gabapentin or carbamazepine may be used for intensive care unit pain control during the acute phase of GBS
- Simple analgesics or nonsteroidal anti-inflammatory drugs (NSAIDs) may be tried, but they often do not provide adequate pain relief.
- Appropriate narcotic analgesics may be used but require careful monitoring for adverse effects in the setting of autonomic denervation.
- Epidural morphine also can be useful
- For the long-term management of neuropathic pain, tricyclic antidepressants, tramadol, gabapentin, carbamazepine, or pregabalinmay be useful
Disease Modifying Treatment
- The main modalities of therapy for Guillain-Barré syndrome (GBS) include plasma exchange (also called plasmapheresis) and administration of intravenous immune globulin (IVIG)
- Treatment shortens the time to walking independently by 40 to 50 percent.
- The precise mechanism of action for IVIG in GBS is unknown but may include providing anti-idiotypic antibodies, modulating expression and function of Fc receptors, interfering with activation of complement and production of cytokines, and interfering with activation and effector functions of T and B cells
- Aside from plasma exchange and IVIG, no other pharmacologic agents have been found to be effective for GBS
- Once the mainstay of therapy for GBS, glucocorticoids have not been shown to be beneficial and no longer have a role
IVIG Dose and duration
- Intravenous immune globulin is given for five days at 0.4 gram/kg per day.
- Patients with more severe clinical disease may benefit from longer duration of IVIG treatment
- Side effects include aseptic meningitis, rash, acute renal failure (mostly related to sucrose containing products), and (rarely) hyperviscosity leading to stroke.
- IgA deficiency can lead to anaphylaxis
- Relapses with increased weakness occur in up to 10 percent of patients with GBS [48,49]. Relapses are usually treated with a partial or complete repeat course of the initial plasma exchange or IVIG treatment
1. www.uptodate.com
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