A to Z of syndromes

A-Z of syndromes: Guillain-Barré syndrome

Signs, symptoms and management of a rare but serious syndrome that affects children.

Signs, symptoms and management of a rare but serious syndrome that affects children.

Guillain_Barre
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Individuals with Guillain-Barré Syndrome (GBS) have sudden-onset polyneuropathy. This is characterised by muscle weakness, which occurs because of damage to the peripheral nervous system. The syndrome is rare but serious, occurs in all races and affects slightly more boys than girls.

The syndrome was named after two French doctors with surnames Guillain and Barré. Its exact cause is unknown, but about 60% of cases occur following infection. These infections can be mild, and viral and bacterial organisms have been implicated. Recently, a link with Zika virus has been made

GBS has also been shown to be associated with the use of vaccinations, such as those for influenza and meningitis, and as uptake in vaccination has increased so has the number of GBS cases, which is why children’s nurses need to be vigilant. The condition is an autoimmune disorder, which involves self-destruction of an individual’s defence mechanism leading to damage of the myelin sheath that covers the nerves. 

Symptoms

The symptoms of GBS usually develop between a few hours to a fortnight. During the acute phase, initial symptoms can include irritability, behavioural changes, paraesthesia, unusual sensations, and pain in the arms and legs. These are often followed by weakness of the limbs and changes to the way the child usually mobilises. In severe cases there may be muscular weakness that extends to the child’s facial muscles. If the muscles of the diaphragm are affected, the child' s respiratory function will be impaired.

Diagnosis is usually based on history, clinical assessment and investigations to eliminate other causes of the symptoms. Children with GBS require admission into an acute care facility and, because progression of the condition is unpredictable, their families should be prepared for possible admission into intensive care. During this time of uncertainty, they will need considerable support and some may need access to counselling   

There is no cure for GBS and the use of steroids is controversial so treatment is supportive and may commence with administration of intravenous immunoglobulins or plasmapheresis. Some children may require mechanical ventilation and so require intensive care. If the damage extends to the child’s autonomic nervous system, there may be abnormalities in the child’s heart rate and blood pressure that require complex supportive therapy. 

Nursing care

Children with GBS require diligent nursing care with particular attention to tissue viability and avoidance of complications of immobility, including hospital-acquired pressure ulcers. They also require input from allied health professionals, including dieticians, to meet their nutritional needs; physiotherapists for intensive chest and limb physiotherapy; and speech and language therapists if they are self-ventilating.

The prognosis is usually good, but in about 5% of cases the children die from complications. Some children may have some mobility and respiratory problems.  

Case study

Maria, aged 8, presented with her mother at a GP having returned from a trip to a theme park abroad a fortnight earlier. Maria had a vague history of feeling unwell, complaining of pins and needles in her legs, a limp and not being as active as usual. Her GP thought there may be muscle weakness and referred the family immediately to an assessment unit at a district general hospital.

The admitting consultant suspected that Maria had Guillain-Barré Syndrome (GBS) and her referral to a regional intensive care unit was discussed in case supporting respiratory therapy was needed. The consultant made it clear that Maria would undergo lots of tests to rule out other conditions. The family were shocked that, having just enjoyed a holiday, she should need such support.

Maria was not in respiratory distress, emergency transfer was not required and the transport team allowed the mother to travel with her. At the unit, Maria’s diagnosis of GBS was confirmed. She was commenced on intravenous immunoglobulin and consented to receive plasmapheresis if required. She had several weeks on high dependency and required tube feeding, but did not need elective intubation and ventilation.

Maria had lots of messages of support from family and friends, and her school teacher sent her audio books so she could keep up to date with what her classmates were reading.

As part of her recovery she had physiotherapy and was transferred back to the referral unit, which was closer to her home and family.

 


Further resources


About the authors

Doreen Crawford is a nurse adviser with healthcare consultancy Crawford McKenzie

Annette Dearmun is divisional head of governance and nursing (children and women’s division), Oxford University Hospitals NHS Trust

Both are consultant editors, Nursing Children and Young People

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