Guillain-Barré syndrome is a rare disorder of the nervous system that may affect between 40 and 80 New Zealanders each year. Symptoms include muscle weakness or paralysis of limbs, the face and respiratory system. It can be a fatal condition but most people with the syndrome will make a complete recovery.
Guillain-Barré (pronounced ghee-yan bah-ray) can affect anyone but men are more commonly affected than women. Its incidence increases with age and it is most common in those aged 50 – 74 years.
Guillain-Barré syndrome is an autoimmune disorder, where the body’s immune system attacks its own tissues. In Guillain-Barré syndrome, immune cells attack the myelin sheath - the fatty substance covering nerve fibres. The myelin sheath insulates and protects the nerve fibres and assists with the transmission of electrical impulses throughout the nervous system. If the myelin sheath is damaged, messages from the brain may be slowed or blocked completely.
What causes the autoimmune reaction that leads to Guillain-Barré syndrome is unknown. However in approximately 60% of cases a viral respiratory infection or a gastrointestinal upset precedes the onset. The organism Campylobacter jejuni which causes diarrhoea
, the Epstein-Barr virus which causes glandular fever
, HIV which is the virus that causes AIDS, and cytomegalovirus (CMV)
have been specifically implicated. It is thought that these viruses may change the nature of nerve fibres so that they appear foreign to the immune system. In rare cases, Guillain-Barré syndrome can be triggered by surgery or immunisation.
Guillain-Barré syndrome is described as a polyneuritis - meaning that many nerves become inflamed. In particular, it affects peripheral nerves - those that connect the brain and spinal cord to the muscles and skin. There are a number of different sub-types of Guillain-Barré syndrome, each affecting the body in a slightly different way.
Signs and symptoms
Guillain-Barré syndrome is an acute condition and has a rapid onset in comparison to other neurological conditions. Signs and symptoms may develop over the course of hours or days, or may take up to three to four weeks to develop.
The first symptoms are usually tingling (pins and needles) or numbness starting first in the feet and rising to the hands and sometimes the face. This is usually followed by varying degrees of muscle weakness of the legs, and then the arms and face. The person may have difficulty holding or gripping objects and their limbs may feel heavy. These symptoms may be vague at first but have a tendency to progress quickly.
As the condition progresses the following signs and symptoms may be experienced:
- Progressive and extreme muscle weakness in the limbs
- Reduced or absent tendon reflexes
- Pain in the muscles and joints
- Paralysis of the eye muscles
- Difficulty breathing.
The severity of symptoms can vary considerably between individuals. Symptoms can range from mild muscle weakness that resolves quickly to complete muscle paralysis.
About 60% of people will be unable to walk when the illness is at its worst. Respiratory function will be impaired in around 50% of cases. In up to 30% of cases the breathing muscles are paralysed to such a degree that a ventilator to maintain breathing is required. Additionally, fluid balance in the body may be unstable, blood pressure may fluctuate, and heart rate may become irregular.
Symptoms tend to be at their worst two to three weeks after their onset. At this stage, there is usually a period of time where the symptoms remain stable. This is referred to as a "plateau" phase and may last for a few days or up to a few weeks.
Diagnosis can be difficult in the early stages of the condition due to the vagueness of initial symptoms. Diagnosis may be easier if the onset is rapid and equal on both sides of the body. To diagnose Guillain-Barré syndrome a doctor will undertake the following:
- A full medical history
- Discussion of current symptoms
- Physical assessment eg: sensation, reflexes and muscle strength
- Blood tests.
Nerve conduction velocity tests may also be recommended. These measure the speed that electrical impulses travel through the nervous system. In Guillain-Barré syndrome, impulses tend to travel more slowly than normal.
A lumbar puncture is also likely to be recommended. This involves inserting a needle into the back and removing a sample of the fluid that surrounds the brain and spine (cerebrospinal fluid). Concentrations of proteins and immune cells in this fluid are likely to be raised in Guillain-Barré syndrome.
There is no cure for Guillain-Barré syndrome. Treatment is largely supportive and aims to minimise symptoms and prevent complications. Hospitalisation is usually necessary in the initial stages, as the course of the condition can be unpredictable. A healthcare team involving doctors, nurses, occupational therapists and physiotherapists will be involved in treatment.
In mild cases rest is important. However, gentle exercises to prevent joints becoming stiff and painful will be recommended. Medications to reduce pain may be required. When the condition is more severe the goal of treatment is to:
- Maintain breathing
- Reduce pain
- Maximise muscle strength
- Maximise joint movement
- Prevent complications of paralysis such as contractures (the shortening and thickening of tendons)
Blood pressure, fluid balance and heart rate and rhythm will also be closely monitored.
Plasma exchange (plasmapheresis)
This treatment involves removing some blood through a needle in the arm or hand. The blood is then separated into plasma and red blood cells. The plasma is discarded and the red blood cells are returned to the body with donated or "clean" plasma. This process may be repeated several times per day over a period of several days. It is thought that plasmapheresis removes antibodies from the body's plasma that may be aiding the autoimmune attack on the nervous system.
Immunoglobulin (gamma globulin) therapy
This is a special protein used naturally by the immune system. When given in high doses intravenously (through a drip into the bloodstream) it is thought to help reduce the autoimmune attack on the nervous system.
Prognosis / recovery
The time it takes to recover from Guillain-Barré syndrome is variable. It may take a few weeks or up to several months, with the average recovery time being three to six months.
The prognosis for the majority of people will be a complete recovery, while 20-30% of people will be left with some degree of weakness and discomfort. In some cases, small improvements may continue to be seen for one to two years after the initial recovery period.
Approximately 5% of cases of Guillain-Barré syndrome are fatal. This is usually as a result of heart rhythm problems but can also be due to respiratory failure, pulmonary embolism
(blood clots in the lungs), or infection.
Support and information
The Guillain-Barré Syndrome Support Group NZ Trust is able to provide further information and support. Go to www.gbsnz.org.nz
The National Office of the Muscular Dystrophy Association (MDA) can also provide more information on Guillain-Barré Syndrome particularly on aspects of managing the condition:
Muscular Dystrophy Association of New Zealand, National Office
Freephone: 0800 800 337
GBS Support Group (date not stated). Guillain Barré syndrome (Web Page). Nelson: Guillain Barré Syndrome Support Group NZ Trust. http://www.gbsnz.org.nz/guillain_barre_syndrome [Accessed: 28/07/17]
Mayo Clinic (2016). Guillain-Barre syndrome (Web Page). Rochester, NY: Mayo Foundation for Medical Education and Research. http://www.mayoclinic.org/diseases-conditions/guillain-barre-syndrome/basics/definition/con-20025832 [Accessed: 28/07/17]
National Institute of Neurological Disorders and Stroke (2011). Guillain-Barré syndrome Fact Sheet (Web Page). Bethesda, MD: National Institutes of Health. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Guillain-Barr%C3%A9-Syndrome-Fact-Sheet [Accessed: 28/07/17]
NHS Choices (2017). Guillain-Barré syndrome (Web Page). Redditch: National Health Service (NHS) England. http://www.nhs.uk/Conditions/Guillain-Barre-syndrome/Pages/Introduction.aspx [Accessed: 28/07/17]
Last Reviewed - July 2017