From the Guidelines
Guillain-Barré syndrome (GBS) is a rare autoimmune disorder that causes rapid-onset muscle weakness, typically starting in the legs and spreading upward, requiring immediate medical attention and hospitalization.
Definition and Pathophysiology
GBS is characterized by an acute-onset ascending sensorimotor neuropathy, although it can present atypically or as a clinical variant 1. The disease is triggered by an autoimmune response, often following a viral or bacterial infection, leading to demyelination and axonal damage in peripheral nerves 1.
Diagnosis
Diagnosis of GBS can be challenging due to heterogeneity in clinical presentation and the lack of highly sensitive and specific diagnostic tools or biomarkers 1. Electrophysiological studies and cerebrospinal fluid analysis may show abnormal results, but these can be normal, especially early in the disease course 1.
Treatment Recommendations
Treatment recommendations include intravenous immunoglobulin (IVIG) at 0.4 g/kg daily for 5 days or plasma exchange with 5 sessions over 1-2 weeks, as both are equally effective in treating GBS 1. Supportive care is crucial and includes:
- Monitoring respiratory function closely, with mechanical ventilation if necessary
- Providing pain management, such as gabapentin or carbamazepine for neuropathic pain
- Preventing deep vein thrombosis with anticoagulation
- Implementing physical therapy and rehabilitation
Prognosis and Outcome
Most patients with GBS recover within weeks to months, but some may have lasting effects 1. The probability of regaining walking ability can be calculated using the modified Erasmus GBS outcome score (mEGOS) prognostic tool 1. Despite the generally positive prospects, death occurs in 3–10% of cases, and long-term residual complaints, such as neuropathic pain, weakness, and fatigue, are common 1.
Special Considerations
In low-income and middle-income countries, treatment options may be limited due to cost, and alternative approaches, such as small volume plasma exchange, may be considered 1. Recurrent episodes of GBS are rare, affecting 2–5% of patients, and prior GBS is not a strict contraindication for vaccination, although discussion with experts may be useful in certain cases 1.
From the Research
Definition and Overview of Guillain-Barré Syndrome (GBS)
- Guillain-Barré Syndrome (GBS) is an acute polyneuropathy characterized by acute flaccid paralysis with or without sensory/autonomous nerve dysfunction 2.
- It is an acute inflammatory disorder of the peripheral nervous system thought to be due to autoimmunity 3.
- GBS can be subdivided into several forms, including acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and Miller-Fisher syndrome (MFS) 4.
Clinical Presentation and Diagnosis
- The clinical presentation of GBS is highly variable, making diagnosis challenging 5.
- GBS is a clinical diagnosis that can be supported by cerebrospinal fluid examination and nerve conduction studies 5.
- Nerve ultrasound and MRI are potentially useful techniques to diagnose inflammatory neuropathies 5.
- Pain can be a confusing symptom in making the diagnosis, especially when it precedes the onset of weakness 4.
Treatment and Management
- Intravenous immunoglobulin (IVIg) and plasma exchange (PE) are the cornerstones of treatment for GBS 5, 2, 3.
- IVIg and PE are equally effective in GBS, although IVIg is usually preferred due to its ease of access and lower rates of complications 3, 6.
- Corticosteroids are not effective in GBS when used alone 3, 6.
- Immunotherapies, mechanical ventilation, supportive care, and complication management are crucial in the intensive care unit (ICU) setting for severe GBS cases 2.
Prognosis and Outcome
- Despite current treatment, GBS remains a severe disease, with about 25% of patients requiring artificial ventilation, 20% unable to walk after 6 months, and 3-10% dying 4.
- Prognostic models can guide the selection of patients with a relatively poor prognosis who may benefit from additional IVIg or intensified treatment 5, 4.
- Advances in prognostic modeling have resulted in the development of simple prognostic scales that predict the chance for artificial ventilation and outcome scales that determine the chance to walk unaided after 1,3, or 6 months 4.