From the Guidelines
Guillain-Barré syndrome is a rare autoimmune disorder where the immune system attacks the peripheral nervous system, causing rapid-onset muscle weakness that can progress to paralysis. This condition is characterized by an annual global incidence of approximately 1–2 per 100,000 person-years, with a higher frequency in males than in females and an increasing incidence with age 1. The clinical presentation of Guillain-Barré syndrome is heterogeneous, with patients typically presenting with weakness and sensory signs in the legs that progress to the arms and cranial muscles, although several distinct clinical variants exist 1.
Diagnosis and Treatment
Diagnosis of Guillain-Barré syndrome is based on patient history, neurological, electrophysiological, and cerebrospinal fluid (CSF) examinations, with electrophysiological studies providing evidence of peripheral nervous system (PNS) dysfunction and distinguishing between subtypes of the syndrome 1. Treatment typically involves either intravenous immunoglobulin (IVIG) at 0.4 g/kg daily for 5 days or plasma exchange (5 exchanges over 2 weeks), with both treatments being equally effective and most effective when started within 2 weeks of symptom onset 1.
Supportive Care and Management
Supportive care is crucial, including respiratory monitoring, as approximately 20% of patients require mechanical ventilation, and physical therapy should begin early to prevent complications 1. Pain management often involves medications such as gabapentin or carbamazepine. The condition results from an immune response that mistakenly attacks nerve coverings, often triggered by a previous infection like Campylobacter jejuni, cytomegalovirus, or Zika virus 1. Prompt diagnosis and treatment significantly improve outcomes, with mortality rates estimated at 3–10% for patients with Guillain-Barré syndrome, even with the best medical care available 1.
Prognosis and Recovery
Most patients recover, though recovery can take months to years, with about 60–80% of patients able to walk independently 6 months after disease onset, with or without treatment 1. Guillain-Barré syndrome is a monophasic illness, although some patients can deteriorate after first stabilizing or improving on therapy, and relapses can occur in 2–5% of patients 1. The key to improving outcomes in Guillain-Barré syndrome is prompt diagnosis and initiation of treatment, emphasizing the importance of early recognition and management of this condition.
From the Research
Definition and Characteristics of Guillain-Barré Syndrome
- Guillain-Barré syndrome (GBS) is an acute polyneuropathy characterized by acute flaccid paralysis with or without sensory/autonomous nerve dysfunction 2.
- It is also described as an acute polyradiculoneuropathy with symptoms that may vary greatly in presentation and severity, including weakness, sensory disturbances, cranial nerve involvement, respiratory insufficiency, autonomic dysfunction, and pain 3.
- GBS is a potentially serious, acute, paralysing, probably autoimmune disease caused by inflammation of the peripheral nerves 4, 5, 6.
Diagnosis and Treatment
- The diagnosis of GBS can be supported by a history of recent diarrhoea or respiratory infection, CSF examination, electrodiagnostic testing, and testing for anti-ganglioside antibodies in certain cases 3.
- Treatment options include intravenous immunoglobulin (IVIg) and plasma exchange (PE), which have been shown to hasten recovery in severe cases 2, 3, 4, 5, 6.
- The European Academy of Neurology/Peripheral Nerve Society Guideline recommends IVIg 0.4 g/kg for 5 days or a course of PE 12-15 L in four to five exchanges over 1-2 weeks for patients unable to walk unaided within 2-4 weeks after onset of weakness 3.
Management and Prognosis
- Intensive care and treatment of severe GBS are crucial, with a focus on mechanical ventilation, supportive care, and complication management 2.
- The modified Erasmus GBS outcome score (mEGOS) and the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) can be used to assess outcome and the risk of requiring artificial ventilation 3.
- Flow charts can assist in making clinical decisions on diagnosis, treatment, and the need for intensive care unit admission 3.