Guillain-Barré syndrome

Guillain-Barré syndrome is an uncommon condition in which a person's own immune system damages the peripheral nerves — the ones running from the spinal cord out to the muscles and the skin. It usually follows one to three weeks after an infection, most often a bout of gastroenteritis or a respiratory illness. It affects roughly one or two people in every hundred thousand a year and can happen at any age. The essential thing to know in advance is that it moves quickly, over days, and that the weakness climbs from the feet upwards: what counts here is not a diagnosis made at home but help called in time. With hospital treatment most people recover, but this illness leaves no room for waiting to see.

How it begins

Almost always the feet and hands go first: tingling, numbness, a sensation of pins and needles or of the limb being padded. Within hours or days weakness joins in — the legs give way on stairs, getting out of a chair is hard, objects slip out of the hands.

Several features let a doctor recognise it.

• It is symmetrical: both sides are affected to much the same degree, not one arm or one leg.
• It travels upwards: from the feet to the thighs, then to the trunk, the arms and the face.
• It is fast: it worsens over days and weeks rather than months, and usually reaches its worst point by the end of the second to the fourth week.
• Reflexes disappear: the doctor taps the knee and the tendons and gets no response, which is one of the signs that convinces them most.
• It hurts: in many people the pain is severe, burning or shooting, most often in the lower back and legs, and it frequently comes before the weakness. It is worse at night.

Later there may be double vision, drooping eyelids, weakness of the facial muscles on both sides, a nasal voice and choking on swallowing. Set apart is a rare variant that starts the other way round, at the eyes: double vision, unsteadiness on the feet and the same loss of reflexes.

When it cannot wait until tomorrow

The danger comes not from weak legs in themselves but from the weakness reaching the muscles that run breathing and swallowing. It does not happen to everyone, but when it happens it is quick and cannot be predicted.

Call an ambulance — across Europe the emergency number is 112 — if any of these appear:

• breathlessness, fast shallow breathing, being unable to finish a sentence in one breath;
• breathlessness on lying flat, so that sleeping has to be done sitting up;
• difficulty swallowing, choking on saliva or water, saliva pooling in the mouth;
• weakness increasing hour by hour: walking in the morning, unable to stand by the evening;
• arms or legs that have stopped moving;
• fainting, sharp swings in blood pressure, a very fast or very slow heartbeat.

If there is weakness and numbness but none of the above, it still needs seeing to, and on the same day: at an emergency department or with a doctor who can examine you straight away. This is not a situation for an appointment next week.

A word about stroke, because the signs are easy to confuse. If the face has dropped on one side, one arm will not lift and speech has become slurred, and all of it came on suddenly, within minutes, the first thought is stroke and an ambulance is called at once. The difference is that Guillain-Barré syndrome takes both sides and unfolds over days. But there is no need to work that out yourself: both call for an ambulance.

Where it comes from

The trigger is nearly always an infection one to three weeks before the first symptoms. The commonest is campylobacter gastroenteritis, the same one that causes diarrhoea after undercooked chicken. Less often it follows influenza, cytomegalovirus, Epstein-Barr virus, mycoplasma, Zika virus or coronavirus infection.

What happens next is a case of mistaken identity. The coat of certain bacteria resembles the surface of nerve fibres, and the antibodies raised against the microbe attack the wrong target. The person does not become infectious: the condition itself is not passed on.

Vaccines come up often, and the answer is worth giving in full. An association has been described, but it is a very weak one, on the order of isolated cases per million doses. At the same time the infections that vaccines prevent cause the syndrome many times more often than the vaccines do, so declining vaccination raises the risk rather than lowering it. For someone who has already had the syndrome, particular vaccines are worth discussing with their doctor — this is one of the uncommon situations where the decision is made case by case.

Anyone can develop it, though the likelihood rises somewhat with age and men are affected slightly more often than women. Nothing a person did or failed to do brings it on: it cannot be prevented.

How the diagnosis is confirmed

The foundation is the neurologist's examination: how the weakness is distributed, whether the reflexes are there, what has happened to sensation and to the facial muscles. The diagnosis rests first of all on that picture, and the tests confirm it and rule out other causes.

• Nerve conduction studies and electromyography, which measure how signals travel along the nerves and how the muscles work. They show where conduction is disturbed, though in the first days they can still be normal, and that does not overturn the diagnosis.
• Lumbar puncture: the spinal fluid shows a raised protein with a normal cell count. In the first week the protein is often still normal, so the test is sometimes repeated.
• Blood tests, to exclude other causes of weakness, including metabolic disturbances and deficiencies.
• Measurement of breathing strength. It is repeated several times a day, because it is the earliest warning that a ventilator will be needed.
• A scan of the spine, if the picture is atypical and compression of the spinal cord has to be ruled out.

What happens in hospital

Treatment always takes place in hospital and often in intensive care — not because the person is already critically ill, but because that is where breathing, swallowing and heart rhythm are watched without a break.

There are two main treatments and they are equally good: intravenous immunoglobulin, or plasma exchange, in which the antibodies are removed from the blood. The choice depends on the person's condition, other illnesses and what the hospital can offer. Both work better the earlier they are started, which is another reason not to put off seeking help. There is no point in combining them.

One thing is worth saying because it surprises people: steroids do not help in this illness. That has been tested in trials, and they should not be given alongside the main treatment, even though they are used in other conditions with similar-sounding names.

Everything else is support, and how much is needed depends on severity: mechanical ventilation if the breathing muscles fail, feeding through a tube if swallowing is unsafe, medicines to prevent clots and compression stockings while movement is limited, and pain relief. Nerve pain responds poorly to ordinary painkillers, so other families of drugs are used — anti-epileptic medicines and antidepressants, at doses the doctor works out. Early physical rehabilitation begins on the ward, without waiting for discharge.

Recovery and what may be left behind

Recovery runs in reverse order: whatever failed last comes back first. It is slow and measured in months. Most people begin walking unaided within the first six months, and full recovery takes anything from a few months to a year or longer.

Some people are left with something permanent: numbness or tingling in the hands and feet, muscle weakness, easy fatigue, nerve pain, clumsiness with fine movements and, for a few, the need for support when walking. Fatigue is the thing most often underestimated: the person looks well from the outside but runs out by the middle of the day, and that has to be allowed for when going back to work. Severe outcomes are rare but real — the illness can be fatal, more often in older people and in those who needed a ventilator.

After discharge there are usually several reviews over the first year: sessions with a physiotherapist for strength and balance, occupational therapy for everyday tasks and, where needed, psychological support, since anxiety and low mood after an illness like this are common and treatable. Recurrence is uncommon, but if weakness returns or increases after a clear improvement, contact the doctor at once: sometimes it means another course of treatment is needed, and sometimes that the condition has turned into a chronic form, which is handled differently.

Online consultation

A remote appointment belongs not to the acute phase but around it. Beforehand, when numbness and weakness have appeared and it is not clear what they are: the doctor will go through how it started, in what order it spread, whether there was an infection in the days before, and will say plainly whether to go to the emergency department now or to arrange tests in the usual way. After discharge, video is convenient for the long tail of questions: what to do about nerve pain, why the energy only lasts half a day, when driving and work can start again, how to pace effort without sliding backwards, what to do about numbness that will not go. The same appointment covers which signs should worry you in future, and the vaccine question. The one thing it cannot do: the acute signs listed above are not assessed remotely — with difficulty breathing or swallowing, call an ambulance.

This material is for information only and does not replace medical advice.