Guillain-Barré syndrome (GBS) is a rare disorder of the peripheral nervous system. The syndrome is named after the French physicians Georges Guillain and Jean Alexandre Barré, who described it in 1916. It is also called acute inflammatory demyelinating polyneuropathy (AIDP).
GBS is an autoimmune disorder in which a person’s own immune system attacks healthy nerve cells in peripheral nervous system. The syndrome can affect the nerves that control muscle movement and nerves that transmit feelings of pain, temperature and touch. This leads to muscle weakness, numbness, and tingling and sometimes paralysis.
Most people recover fully from GBS, but some people have long-term nerve damage. 3%-5% of GBS patients may die from complications, which include paralysis of the muscles that control breathing, blood infection, lung clots or cardiac arrest.
The cause of this condition is unknown, but it’s typically triggered by an infectious illness, such as the gastrointestinal infection or a lung infection. Few countries from Europe and Asia have reported familial occurrence of GBS (42 GBS patients found from 20 families). In a hospital based study from South India among 150 GBS cases reported during 10 years period, 2 cases belonged to a single family.
According to WHO overall incidence of GBS is 0.4 to 4.0 people per 100 000 per year. People of all ages can be affected, but it is more common in adults and in males.
During 2015 and 2016, nine countries and territories(El Salvador, French Polynesia, Martinique, Colombia, Suriname, Puerto Rico, Brazil, Panama, Venezuela (Bolivarian Republic of) have reported an increased incidence of Guillain-Barré syndrome (GBS) and/or laboratory confirmation of a Zika virus infection among GBS cases. Researchers are studying a potential link between the surge in GBS cases and Zika virus infection. Zika virus is not yet proven to be the cause of the increased GBS incidence in Brazil, Colombia, El Salvador, Suriname or Venezuela.
Symptoms usually start with numbness or tingling in the fingers and toes. Over several days, muscle weakness develops in the legs and arms. The lower limbs are usually involved before the upper limbs. Weakness is ascending and symmetrical in nature. The weakness may progress to the arms, face (In 45%-75% of patients III-VII and IX-XII cranial nerves are involved), and muscles of respiration (the chest muscles are affected in 20%-25% of cases).
The symptoms of Guillain-Barré syndrome include:
- tingling or prickly sensations in fingers and toes
- muscle weakness in legs that travels to upper body and gets worse over time
- difficulty in walking steadily
- difficulty in moving eyes or face, talking, chewing, or swallowing
- severe lower back pain
- loss of bladder control
- fast heart rate
- difficulty in breathing
Most people recover fully from the disease; however some cases continue to experience weakness.
The etiology of GBS is not completely understood. It is not a contagious disorder.
About two-thirds of people with GBS develop it soon after they have been suffered with diarrhea or a respiratory infection. This suggests that the disorder may be triggered by an improper immune response to the previous illness.
Infections that may trigger GBS include:
- Campylobacter jejuni which can cause gastrointestinal infection. This bacterium is one of the most common risk factors for GBS.
- Cytomegalovirus (CMV)
- Epstein Barr virus
- Varicella zoster virus
- Human immunodeficiency virus (HIV), dengue, or influenza
- Less commonly vaccinations, surgical procedures, and trauma have been reported to trigger the development of GBS.
GBS can affect person of any age group, however the incidence of GBS increases with age. People older than 50 years are at greatest risk for developing GBS.
Guillain-Barré syndrome is difficult to diagnose because the symptoms are similar to other neurological disorders. Diagnosis is based on symptoms, findings on neurological examination including diminished or loss of deep-tendon reflexes.
Routine blood tests are done, to exclude other diseases with similar symptoms and for better assessment of functional status and prognosis.
Specific tests are required to identify the cause of trigger of GBS.
The following tests are used to confirm a diagnosis of GBS-
- Lumber puncture (Spinal tap) – People with Guillain- Barré syndrome has higher-than-normal levels of protein in their cerebrospinal fluid without an elevation in white blood cells. The increase in cerebrospinal fluid (CSF) protein reflects the widespread inflammation of the nerve roots.
- Electromyography-An electromyography is a nerve function test. It reads electrical activity from the muscles and distinguishes whether muscle weakness is caused by nerve damage or muscle damage.
- Nerve Conduction Tests-Nerve conduction studies may be used to test how well nerves and muscles respond to small electrical stimuli.
- Imaging studies, such as magnetic resonance imaging (MRI) and computed tomography (CT) scanning of the spine may be helpful in excluding mechanical causes of myelopathy.
On 25th February 2016, under Identification and management of Guillain-Barré syndrome in the context of Zika virus, WHO has recommended using Brighton criteria as the case definition of GBS, so that standardized information may be available for epidemiologic purposes(not as a criterion for treatment). This criterion is based on presenting clinical findings and ancillary testing including neurophysiology and lumbar puncture findings. Patients are categorized as level-1 (the highest level of diagnostic certainty) to level -3(the lowest level of diagnostic certainty)
The goal of treatment is to lessen the severity of symptoms and maintain vital functions normal while nervous system recovers.
- GBS patients are usually hospitalized so that they can be monitored closely.
- A multidisciplinary approach is required in the acute phase, combining supportive and disease-modifying therapy (plasma exchange or high-dose immunoglobulin [IVIG]).
- Respiratory management-Respiratory status should be monitored in all patients. Up to 30% of patients need ventilatory support or airway protection.
- Cardiovascular management-Haemodynamic monitoring of pulse and blood pressure should be started early. Hypotensive / Hypertensive episodes may be managed accordingly.
- Prophylaxis for prevention of deep vein thrombosis (DVT)- There is increased risk of DVT (formation of blood clots) in DBS patients due to immobility and hypercoagulability (increased tendency toward blood clotting from treatments such as intravenous immunoglobulin).Medication and support stockings may be used in non-ambulatory patients until they are able to walk independently.
- Pain management-Safe and effective drugs are required for pain management in acute phase.
(b) Immunotheraphy: Immunotherapy comprises IVIG or plasma exchange.
- Intravenous immunoglobulins (IVIG) – High doses of immunoglobulin can help to block the antibodies causing GBS. (Immunoglobulin contains normal, healthy antibodies from donors). It should begin within two weeks from the onset of symptoms.
- Plasmapheresis (Plasma Exchange) – Plasmapheresis is a process that filters the blood and removes harmful antibodies. Plasma exchange is most beneficial when initiated within 7-14 days from the onset of the disease.
(c) Rehabilitation services:
- Rehabilitation is started in the form of physiotherapy in the acute phase of the disease. It comprises gentle strengthening involving isometric, isotonic, isokinetic, and manual resistive and progressive resistive exercises. It should be focused on proper limb positioning, posture, orthotics, and nutrition.
Most people recover fully from GBS, but some people have long-term nerve damage. Residual weakness can be seen in 30% of GBS cases after three years. About three percent of cases may suffer a relapse of muscle weakness and tingling sensations many years after the initial attack. 3%-5% of GBS patients may die from complications, which include;
- paralysis of the muscles that control breathing,
- sepsis (blood infection),
- pulmonary embolism (lung clots),
- cardiac arrest.
Guillain-Barré syndrome is a condition (not a disease itself), and its causation is not known exactly. Therefore no specific preventive measure can be indicated.
Sometimes vaccination may trigger the occurrence of GBS; hence vaccination is not suggested in acute phase and up to a period of one year after an episode of GBS.
As there is increase in cases of Guillain-Barré syndrome in areas where Zika virus is circulating and researchers are investigating to prove the link between the two, WHO recommends that anyone living in or traveling to areas where the Zika virus is circulating take precautions to avoid mosquito bites.
When possible, patients should be treated in an intensive care unit in order to keep continuous monitoring and to respond immediately to any urgency. Thus complications due to disease and immobility can be identified and responded early by healthcare workers.
Patients of Guillain-Barré syndrome suffer not only physical difficulties but emotionally painful periods also. Residual symptoms may lead to long term disability and cause difficulties in attaining prior lifestyle or occupation. Rehabilitation services combined with psychological counseling would be helpful in recovery of the patients.
a Aik KR et al, Familial Guillain-Barré syndrome: First Indian report. Ann Indian Acad Neurol [serial online] 2012[cited 2016 Mar 10]:15:44-7.available from .annalsofian.org/text.asp