An autoimmune disease

Guillain-Barre syndrome develops when the body’s immune system attacks and damages the body’s own tissues. If you have Guillain-Barre syndrome, your immune system targets myelin, the protein that insulates and protects your main nerves. Myelin helps electrical impulses to travel along the nerves and without it nerve transmission can become very variable.

Guillain-Barre syndrome can also be called acute inflammatory demyelinating polyneuropathy:

• Acute: because it tends to get better within a relatively short time.
• Inflammatory: because it involves inflammation of the nerves.
• Demyelinating: because it involves loss of myelin.
• Polyneuropathy: because it damages many nerves.

What causes Guillain-Barre syndrome?

The exact cause is not really known. It often starts after a minor infection of the lungs or gastrointestinal tract. The development of Guillain-Barre syndrome has been definitely associated with:

• Bacterial infections
• Viral infections including cytomegalovirus, Epstein–Barr virus, herpes simplex virus, and HIV Systemic lupus erythematosus Hodgkin’s disease

Which nerves does the syndrome affect?

Guillain-Barre syndrome usually affects nerves outside of the brain and spinal cord, which form the peripheral nervous system. The peripheral nerves include those that carry sensory information from the body to the brain (the sensory neurons) and those that carry information from the brain to the muscles and cause movement (the motor neurons). Depending on the types of nerve affected, the symptoms can vary.

The impact of Guillain-Barre syndrome

The symptoms of Guillain-Barre syndrome usually begin two to four weeks after having a mild infection. One of the first signs is getting pins and needles and feeling generally weak, particularly in the arm and leg muscles. Muscle weakness can then spread quickly, over hours or days, to affect other muscles.

Typical symptoms of Guillain-Barre syndrome once it has fully developed include:

• Loss of reflexes in the arms and legs
• Low blood pressure (hypotension), which can cause fainting
• The feeling that your muscles ‘won’t work’ Numbness
• Muscle pain, which can be like cramp
• Not being able to coordinate your arms and legs properly

Guillain-Barre syndrome can also affect the eyes and face, leading to blurred vision and facial paralysis. In some people, it also causes weakness in the heart muscles, leading to palpitations.

In the most serious cases, people find that the muscles that control their lungs become weak and this can affect their breathing. If this happens, rapid medical attention is important to avoid respiratory distress.

Because the symptoms of Guillain-Barre syndrome can have such potentially severe consequences, you might need to go into hospital for a while during your illness, so that your condition can be monitored carefully.

Who gets Guillain-Barre syndrome?

Guillain-Barre syndrome is relatively rare, certainly nowhere near as common as Bell’s palsy. Globally, the syndrome affects just 1.1–1.8 people out of every 100,000. Anyone of any age can be affected, but this autoimmune disease is most common between the ages of 15 and 35 and 50 and 75, and affects slightly more men than women.

Diagnosing and treating Guillain-Barre syndrome

Guillain-Barre syndrome is usually diagnosed after tests have ruled out other illnesses that have similar symptoms. There is no cure, so treatment usually aims to reduce the symptoms:

Plasmapheresis: if Guillain-Barre syndrome is identified early enough, within 1–2 weeks of first noticing symptoms, plasmapheresis has been shown to shorten recovery times significantly. This involves passing the patient’s blood through a machine that filters and ‘cleans’ it to remove the antibodies that are attacking the nerve cells.

Intravenous immunoglobulin therapy (IVIG): this can also block anti-myelin antibodies. IVIG treatment can be just as effective as plasmapheresis, but can be started up to 4 weeks after symptoms first appear.

Anti-coagulant drugs help prevent blood clots. Ventilation to help with breathing if the lung muscles are affected. Anti-inflammatory drugs and analgesics to help reduce swelling and pain.

After-effects of Guillain-Barre syndrome

About 85% of people who develop Guillain-Barre syndrome make a full recovery, but this can take from 6 to 18 months. A third of people affected find that their muscle weakness persists for three years.

Between 7 and 15% have permanent muscle weakness. In the most serious cases, the after-effects of Guillain-Barre syndrome can lead to a loss of mobility, having to use a wheelchair and not being able to work or get employment. Early diagnosis and prompt supportive treatment is therefore essential.