Introduction – What is Guillain-Barré Syndrome?
Guillain-Barré Syndrome – often referred to as GBS, is when the body’s own immune system attacks parts of the nervous system.
At present there is no known cause for GBS however, two-thirds of patients have influenza, respiratory illness or gastroenteritis prior to GBS. This can be up to 6 weeks before the onset of the neurological symptoms but typically around 1-3 weeks. Other triggers that have been linked to GBS are trauma, surgical procedures, immunisations and vaccinations, malignancy and HIV.
At present, there is also a lot of research being carried out as to whether the Zika virus may also be a trigger for GBS and other viral infections such as dengue, chikungunya and Japanese encephalitis.
This infection then leads to the immune system attacking nerves. It is not an inherited disease nor is it contagious but simply a spontaneous event that happens as a side-effect of that initial illness.
The syndrome was named after the French Neurologists George Guillain and Jean Alexandre Barré.
What are the symptoms?
For some people, the symptoms start in the feet and hands before passing up the legs and arms. In other cases it can start at the neck and move downwards. The patient would get pain, tingling and numbness before it then spreads to increasing muscle weakness and eventually coordination issues before paralysis in various degrees may occur. In some cases the paralysis can affect the breathing, blood pressure or heart rate and in these cases it can be considered a medical emergency. Death can occur with GBS.
Patients will often be put on ventilators to assist with their breathing and watched closely for problems such as abnormal heart beats, blood clots, high blood pressure, low blood pressure and infections.
After being initial diagnoses the symptoms then progress over the course of hours, days or weeks. Most patients reach a peak within the first 2 weeks after symptoms appear.
Each patient will react differently, with some having:
There is not a known cause for GBS at present but what scientists do know that it triggers the body’s own immune system to attack itself which is referred to as an autoimmune disease.
The cells in the body usually only attack foreign material and organisms such as bacteria and viruses however in GBS the immune system starts to attack the nerves and in particular the myelin sheath that coats the outside of the nerves. Humans require the myelin sheath around a nerve to help speed up the transmission of nerve signals over long distances such as down the length of a body. If the myelin sheath is damaged then the nerves are not able to transmit as well and the muscles then lose their ability to respond to the brain’s commands. The brain is also not able to get sensory signals and it means a patient stops feeling textures, heat, pain and other sensations or may receive tingling sensations.
As GBS is usually caused by a bacterial or viral infection the virus may change what the cells look like to the nervous system so they then treat them like they are foreign cells and start to attack them. It could also be that the virus makes the immune system itself less discriminating about what cells it sees as its own and also the lymphocytes and macrophages to attack the myelin sheath.
A lot more research needs to be done before scientist will fully understand the exact nature of GBS.
There are two main tests that are used by medical professionals to confirm GBS:
1) Nerve conduction studies and electromyography – It is a medical diagnostic test used to evaluate the function, especially the ability of electrical conduction, of the motor and sensory nerves of the human body.
2) Lumbar puncture – a needle is placed into the lower part of the spine and a small amount of cerebrospinal fluid is taken out. This fluid is clear and colourless and is found around the brain and spinal cord. Analysis of the CFS can show a variety of results about the present condition of the brain and spinal cord.
CSF analysis is a vital tool as it may exclude other infectious causes and it should be done early on in diagnosis. However during the first week of the illness the CSF protein may be normal and therefore may need to be repeated. The expected result for a GBS diagnosis would be an elevated CSF protein with a normal cell count.
Other tests may also be carried out to assist in the diagnosis:
Variations of GBS
GBS can come in different forms that have differing levels of paralysis and affect different parts of the body. It is important for medical professionals to diagnose the correct variation to ensure recovery occurs quicker:
AIDP – Acute Inflammatory Demyelinating Polyradiculoneuropathy – progressive weakness of two or more limbs with reduced or absent tendon reflexes. Symptoms start within 4 months. It affects both the proximal and distal muscles. There may also be issues with sensory and motor functions.
AMAN – Acute Motor Axonal Neuropathy – It usually shows as an acute weakness or paralysis without any sensory loss with reduced or absent reflexes. It is more likely to follow a Campblyobacter jejuni (a gastroenteritis bacteria) rather than a respiratory illness. On diagnostic testing it can be differentiated between AIDP due to the selective involvement of motor nerves, preservation of sensory fibres and on electrophysiology showing axonal features. It also has a much faster progression.
It was originally identified in the late 1990s and affects 30-65% of GBS sufferers in Asia, Central and South America.
AMSAN – It is associated with sensory and motor deficits with axonal loss. It often shows as full paralysis and sensory loss often with incomplete recovery. It is usually related to AMAN. It is unknown at present whether IVIG is the correct therapy for AMSAN. Due to the high rate of death, IVIG and PE should always be considered as a preventative measure.
BBE – Bickerstaff’s brainstem encephalitis – It is similar to Miller Fisher Syndrome but there is also altered consciousness or long tract signs or both. A long tract sign is such as muscle spasticity or bladder involvement that usually indicates a lesion in the middle or upper parts of the spine cord or brain. Patients often get drowsiness, hyper reflexia and extensor plantar response.
PCB – Pharyngeal-cervical-brachial – The patient symptoms tend to start from the top downwards and affects possibly only one side of the body but could be both. There is acute arm weakness, issues with swallowing, neck bending and facial weakness. There may also be respiratory issues.
MFS – Miller-Fisher Syndrome – This is shown by impaired eye movements called ophthalmoplegia. It affects 5% of all GBS cases. It mainly affects the lower limbs and damages entire peripheral nerves.
The patient may also suffer from abnormal coordination called ataxia and a loss of tendon reflexes. It tends to not cause limb or respiratory muscle weakness but bulbar and facial palsy may happen and eye abnormalities and ptosis may be present.
It can be a self-limiting condition but there can be an overlap of the BBE or PCB varieties. Recovery is usually good and starts 2-4 weeks from the onset of symptoms with usual complete recovery in 6 weeks. Relapses can occur in 3% of cases.
Acute Pandysautonomia – The patient usually has diarrhoea, vomiting, dizziness, abdominal pain and urinary retention. They may has have a varied heart rate, decreased sweating, salivation and lacrimation (lack of tears.)
Wallerian-like Degeneration – It is seen in rural areas of China in young adults and children. It seems to be more prevalent in the summer months. It has also affected patients in North America and Europe. The symptoms are when a nerve fibre is cut or crushed, leading to the part of the axon which has become separated from the neuron’s all body to degenerate. It can be referred to as anterograde or orthograde degeneration. The patient will have periods of forgetfulness, hollowness in the cranium, anger, irrationality, possible speech impediments and weakness in the knees and ankles.
Patients are usually treated in either a general or neurology ward or may have episodes on an intensive care unit. There is no known cure for GBS but there are therapies which can lessen the severity of the illness and aid recovery.
There are two main treatments available to a patient:
The treatments work by aiming at the antibodies in your immune system to stop them from further damaging your peripheral nerves.
In the UK, IVG is safer and easier to give then plasma exchange but it has been quoted that it costs £5000 per treatment and patients will often need 5 treatments given over a 5 day period although this may vary.
Further treatment such as counselling, physiotherapy, occupational therapy and speech and language therapy may be an option to aid recovery.
Steroids have also been used to try and reduce the severity of GBS but tests have shown that it may be actually be detrimental, not helpful.
The most important part of recovery for a GBS patient is to ensure that the body’s function is maintained at all times. It may mean a patient needs a heart monitor or a mechanical ventilator to assist breathing. Patients are often () put on ICU or HDU wards in hospitals. Due to the paralysis, patients may also suffer from other conditions such as bed sores or pneumonia.
Nurses are therefore encouraged to manually move a patients arms and legs to ensure muscle function remains and prevents deep vein thrombosis.
There are three outcomes for GBS:
GBS recovery does not happen over night and it may be several weeks, months or over a year before recovery happens.
Long term complications can include:
Who is affected?
GBS affects 1,200 people (1 in 100,000) in the UK each year and is more likely to affect men in their 30s to 50s but can affect both sexes.
It can however affect children as young as a few months old but is much rarer.
Notable people affected by GBS are:
Markus Babbel – A former international football player
Tony Benn – A British Politician
Andy Griffith – An American author
Luci Baines Johnson – Daughter of the former American President Lyndon Johnson
There is also some research published in 2003, that President Franklin D Roosevelt had GBS, rather than Polio, which is what he was diagnosed with.
They are the Guillain-Barré Syndrome and Associated Inflammatory Neuropathies charity. They support people affected by GBS, CIDP and other neuropathies. They also fund research for these neurological illnesses.
They can also arrange for patients to speak to medical staff or students for training purposes by emailing firstname.lastname@example.org or for a recovered patient to speak to you if you are in hospital suffering from GBS.
Telephone: 0800 374 803
Assisting family members looking after a child affected by GBS.
Telephone: 0808 808 3555
GBS/CIDP Foundation International
They are the predominant global non-profit organisation supporting individuals and families affected by GBS/CIDP and MMN. Their mission is to improve the quality of life for individuals and their families.
Telephone: (610) 667-0131
Get in touch