35. Guillain Barré Syndrome Flashcards
You are called to see a 25-year-old man on a medical ward with a 10-day
history of progressive weakness.
What is the differential diagnosis?
Guillain–Barr ´e syndrome
Motor neurone disease
Multiple sclerosis
Polyneuropathy – polio, HIV
Hypo/hyperkalaemic periodic paralysis (or iatrogenic).
What is the clinical picture of Guillain–Barr´e syndrome?
This syndrome is characterised by:
Progressive, symmetrical, ascending flaccid motor weakness.
Around 80% have sensory symptoms (pain is very common).
Around 65% have autonomic dysfunction.
It is often preceded by an infective illness.
The age-specific curve shows a bimodal distribution with peaks in young
adults and the elderly. Males are more commonly affected.
The main problems in the acute situation are:
Respiratory failure requiring assisted ventilation due to progression of the
paralysis to involve respiratory muscles.
Autonomic neuropathy which can be severe causing orthostatic
hypotension and cardiac arrhythmias.
Miller–Fisher syndrome
Miller–Fisher syndrome (1956) describes the association of
ophthalmoplegia, ataxia and areflexia.
What are the causes of this syndrome ?
What are the causes of this syndrome ?.
The syndrome may be an immune reaction triggered by either infection or
vaccination.
Antecedent infections include:
Antecedent infections include:
Campylobacter
Campylobacter jejuni, a major cause of bacterial
gastroenteritis worldwide, is the most frequent
antecedent pathogen (up to 45% in some studies)
Cytomegalovirus The second commonest associated infection which
particularly affects young females
Epstein–Barr virus
Mycoplasma
Association with HIV
Pathogenesis
It is likely that immune responses directed towards the infecting organisms are involved in the pathogenesis by cross-reaction with neural tissues.
Possible associated vaccinations include influenza, polio, rabies and rubella.
Most large-scale epidemiological studies have failed to find a cause–effect
relationship. In up to 30% of cases no cause is found.
There is widespread segmental demyelination of peripheral nerves.
Cerebrospinal fluid typically shows few cells with a high protein content (in
90% of cases), though the diagnosis remains a clinical one
Why or when might you intubate these patients?
Around 25% of patients require mechanical ventilation, so close monitoring must be ensured should a deterioration in respiratory function occur.
Forced vital capacity (FVC) should be measured regularly.
There are no ‘rules’ regarding mechanical ventilation.
However:
Some recommend ventilation if the FVC < 1L (others < 10–15 ml/kg)
The speed of deterioration will also influence any decisions
Other factors, e.g. aspiration, bulbar weakness should be taken into account
Do you know of any specific treatments?
Plasma exchange
Involves the removal of about 200 ml/kg of plasma
over 4–6 sessions and replacement with colloid or
crystalloid
Thought to work by removal of a humoral
demyelinating factor. This treatment does not
influence mortality but reduces the ventilation and
complication rate.
Immunoglobulin
Intravenously for 5 days
Much more convenient than plasma exchange
Randomized control trials show that
immunoglobulin and plasma exchange are equally
effective in reducing the time to functional recovery.
Immunosuppressants Steroids and other immunosuppressants are no
longer recommended.
What other problems might they encounter on ICU?
Pneumonia
Line infections
DVT
Nutritional deficits
Psychological
Psychological There should be early and active psychological support
for the patient and relatives.
Autonomic neuropathy
Ensure adequate circulating volume and sedation.
β-blockers, atropine and pacemakers have all been
used to manage autonomic disturbances.
Pain
Pain is common (especially in the back and lower
limbs) and can be a major problem. Opiates are often
required. A randomized, double-blind cross-over trial
showed that carbamazepine is a useful adjuvant for
pain control, reducing narcotic requirements.
Intensive physiotherapy is essential.
GI haemorrhage
Do you know the prognosis?
Around 80% make a near complete recovery, although the speed of recovery is variable.
At least 10% will have a significant permanent disability and the
mortality rate is 5–10%.
Poorer prognosis is associated with:
Older patients
Preceding Campylobacter jejuni infection
Need for mechanical ventilation
Rapid progression of symptoms
Extensive disease
