A GP's Guide to Guillain Barré Syndrome & CIDP
By James G McLeod AO, FRCP (Lond), FRACP
Reprinted from Modern Medicine of Australia October
Guillain-Barré Syndrome is the name given to acute idiopathic polyneuritis
in honour of the French neurologists who described the condition
in two soldiers in 1916. Undoubtedly there had been earlier descriptions.
In brief, it is a clinical syndrome of progressive muscle weakness
and respiratory paralysis associated with absent reflexes, which
develops over a period of three to four weeks, usually following
a viral or other infection.
The annual incidence is about two per 100,000 so that, for example,
about 50 to 60 cases would be seen in Sydney each year. Early diagnosis
is important since death from rapidly developing respiratory paralysis
can occur if the illness is not recognised, and because there are
now specific forms of treatment such as plasmapheresis and intravenous
immunoglobulin that can help speed up recovery, reduce disability
and prevent complications.
Figure 1a and b. Myelinated motor nerve fibres in peripheral
(a) top. Normal myelinated fibre with nerve impulse being conducted
node to node, causing muscle contraction.
(b) bottom. Autoimmune attack on myelin causes a block in nerve
preventing muscle contraction. Plasmapheresis and intravenous
inhibit immune-mediated attack on myelin, allowing remyelination
and nerve conduction to be restored. Illustration for Modern
Medicine by Chris Wikoff
- AETIOLOGICAL FACTORS
- Guillain-Barré Syndrome can affect people of any age, but the
incidence increases with age and is at its highest in the age
group 50 to 74 years. Men are affected more commonly than women.
In about 50 to 60% of cases there is a history of a recent viral
or other infection. Organisms that have been specifically implicated
are Campylobacter jejuni, which causes diarrhoea, and Epstein-Barr
virus, which causes glandular fever. Vaccinations may also precipitate
its onset. HIV infections may cause a syndrome like Guillain-Barré
- Guillain-Barré Syndrome is an autoimmune disease, in which there
is an attack by macrophages and T-cells on healthy myelin in peripheral
and cranial nerves, resulting in a block in conduction of nerve
impulses. The central nervous system is unaffected.
Table 1 - Clinical Features of Guillain-Barré Syndrome
- Progressive symmetrical weakness of the limbs
Paraesthesiae in the hands and feet
Back pain in 30% of cases
Depressed or absent reflexes
Cranial nerves are affected (especially the facial
nerves) in 50% of cases
Progression to peak disability in four weeks
- CLINICAL FEATURES
- The common initial symptoms are numbness and tingling in the
lower limbs (see Table 1). Back pain is a major symptom in about
one third of patients. The sensory symptoms are soon followed
by weakness in the legs and arms and may be more pronounced in
the proximal muscles. Cranial nerves are affected in over 50%
of cases; the facial nerves being most commonly involved. The
acute onset of bilateral facial palsy (in contrast to unilateral
Bell's palsy) should immediately raise the suspicion of Guillain-Barré
Syndrome. Paralysis of extraocular muscles, causing ptosis and
double vision, occurs in about 10% of cases. Weakness in the limbs
may be in a proximal as well as a distal distribution. Tendon
reflexes are usually absent or greatly reduced, and although sensory
loss is usually not profound there may be impairment of sensation
in a glove and stocking distribution. Sphincter disturbances are
present in only a small proportion of cases and should alert the
clinician to the possibility of another diagnosis, such as spinal
cord compression. Progression of disability may continue for a
period of up to four weeks.
- The diagnosis is essentially a clinical one depending on the
history of preceding infection, numbness and tingling, progression
of weakness, back pain, and the findings of facial or limb weakness,
and depressed or absent reflexes. The diagnosis can be difficult
in the early stages because there may be few objective signs.
Because of this many patients are thought simply to be anxious
or hysterical and may be sent home from hospital emergency departments
or doctors' surgeries only to become rapidly weak and develop
respiratory paralysis at home overnight. A high index of suspicion
of the disease is necessary to prevent these tragedies, and the
onus is on the general practitioner to be alert to the possibility
of Guillain-Barré Syndrome.
Other conditions that should be included in the differential diagnosis
of patient with an acute progressive paralysis include spinal cord
lesions, myasthenia gravis, poliomyelitis, acquired hypokalaemia,
periodic paralysis, polymyositis and botulism (Table 2).
Other causes of acute neuropathy such as acute intermittent porphyria,
heavy metals and other toxins, lymphoma, carcinoma of the lung,
vasculitis, diptheria and Lyme disease should be excluded, as should
diabetes and vitamin deficiencies (Table 3).
Table 2 - Other causes of rapidly progressive weakness
- CLINICAL COURSE AND PROGNOSIS
- The interval from onset to peak disability may vary from hours
to weeks. About 30% reach their maximum deficit within seven days;
others progress for up to four weeks.
About 60% of cases are unable to walk at the height of their illness.
Respiratory function is impaired in over half of the patients and
about 20 to 30% will require assisted ventilation. The mortality
rate is about 5%, the most common causes of death being complications
of respiratory failure, pulmonary embolism, cardiac arrhythmias,
autonomic failure and infections.
In about one third of patients the first signs of recovery occur
within two weeks and in about one third recovery begins between
the second and fourth weeks; in the remainder of patients, up to
three months may elapse before definite improvement is evident.
About 70 to 80% of patients make a good recovery with little or
no residual disability. The remainder have varying degrees of distal
muscle wasting and weakness.
- Management of Guillain-Barré Syndrome is outlined in Table 4.
- IMMEDIATE MANAGEMENT BY THE GP
- If the clinical features suggest Guillain-Barré Syndrome, the
patient should be sent immediately to a hospital where respiratory
support is available. If the diagnosis is considered but there
are no abnormal signs the patient should be told to contact the
doctor immediately if weakness or difficulty in breathing develop,
and should be reviewed the following day.
Table 3 - Other causes of acute polyneuritis
Acute intermittent porphyria
Vitamin B deficiencies
Heavy metals and other toxins
- HOSPITAL MANAGEMENT
- Vital capacity should be measured every two to four hours in
the initial stages. Tracheostomy and artificial ventilation may
be necessary. Careful nursing is important, particular attention
being paid to the care of skin, bladder, bowels, mouth, pharynx
and trachea. Bowel and urinary tract infections require prompt
treatment. Intravenous or intragastric feeding may be necessary.
Splints to prevent foot and wrist drop may also be required, and
physiotherapy should be commenced immediately.
- A number of large trials have demonstrated the effectiveness
of plasmapheresis which should be commenced as early as possible,
certainly within the first two weeks; 200 to 250mL/kg should be
exchanged over a 7-14 day period. Albumin or artificial plasma
solutions are used to restore intravascular volume.
- Intravenous human immunoglobulin
- A number of studies have demonstrated that high dose intravenous
human immunoglobulin (0.4 g/kg daily for 5 days) is equally effective,
safer and more readily administered than plasmapheresis. However,
in Australia it is very expensive and difficult to obtain.
- Corticosteroids and immunosuppressive
- There is no evidence that corticosteroids and immunosuppressive
therapy are of any benefit in Guillain-Barré Syndrome.
- PHYSICAL THERAPY
- Early arrangements need to be made for physiotherapy. Some patients
are only mildly affected and make a rapid recovery. Others are
left with residual disability for example, hand weakness, foot-drop
and the need for walking aids. This latter group will require
long term rehabilitation.
- Being completely paralysed and requiring ventilatory support
is a terrifying experience and patients need to be given a full
account of their illness, the likely duration of their paralysis
and the strong likelihood of very good recovery. Special counselling
may be necessary.
- SUPPORT GROUPS
- Guillain-Barré Syndrome support groups exist in most major cities
and are valuable in providing long term assistance to patients.
In the acute stages of the disease patients are usually reassured
by talking to others who have endured the same experience and
made a good recovery.
- HOME MANAGEMENT
- On returning home from hospital, care will be shared between
the specialist, the GP and rehabilitation specialists. The GP
will be responsible for primary care: managing any complications
or recurrent illness, providing support and encouragement to the
patient and family, or coordinating phone care and physiotherapy.
- Nerve conduction studies are usually abnormal in Guillain-Barré
Syndrome, with marked slowing of motor conduction and impaired
sensory conduction. Lumbar puncture is usually performed to confirm
the diagnosis; typically the CSF protein is elevated and white
cell concentration is not increased. Other investigations performed
routinely include full blood count, erythrocyte sedimentation
rate, serum electrolytes, creatinine and urea, blood glucose,
and serological tests for HIV and Lyme disease.
- The presence of a high white sell count in the cerebrospinal
fluid (greater than 30x106/L) should raise the suspicion of HIV
- Table 4 - Management of Guillain-Barré
- Admit to hospital
Monitor vital capacity
Treat respiratory failure:
- artificial ventilation
- Specific therapy:
- intravenous immunoglobulin
- Intragastric or intravenous feeding may be necessary
- General nursing care:
- Prevent deep venous thrombosis:
- subcutaneous heparin 5,000 units b.d.
- walking aids
Psychological support and communication
Guillain-Barré Syndrome support groups
- PRACTICE POINTS
- Guillain-Barré Syndrome should be considered as a diagnosis
in anyone presenting with acute or subacute progressive weakness.
Common initial symptoms are back pain, paraesthesia and
Bilateral facial weakness should alert the clinician to
Respiratory paralysis may develop rapidly and the patient
should be admitted to hospital as soon as the diagnosis is suspected.
Plasmapheresis and intravenous immunoglobulin shorten the
duration of the illness and reduce the frequency and severity
Twenty per cent of patients will have residual physical
disability requiring long-term management and GP supervision at
- VARIANTS OF GUILLAIN-BARRÉ SYNDROME
- Miller Fisher Syndrome is a variant of Guillain-Barré Syndrome,
characterised by opthalmoplegia, ataxia and araflexia without
significant weakness, or sensory symptoms or signs. Like Guillain-Barré
Syndrome it usually follows a respiratory tract infection. The
initial symptoms are ataxia of gait, double vision and headache.
The ataxia is out of proportion to weakness or sensory disturbance.
Treatment is the same as for Guillain-Barré Syndrome.
Acute sensory loss with absent reflexes is another rare
manifestation of acute polyneuritis in which the sensory features
are more prominent than the associated weakness.
Acute autonomic neuropathy (acute pandysautonomia is an
acute auto-immune disorder affecting the autonomic nervous system
and manifested by postural hypotension, impairment of sweating,
lacrimation and bladder and bowel function.
Chronic inflammatory demyelinating polyneuropathy (CIDP)
has a similar pathogenesis to Guillain-Barré Syndrome, but has a
slower onset, usually developing over weeks or months. It may run
a relapsing and remitting or progressive course over many years.
Nerve conduction studies are abnormal. The condition usually responds
to treatment with corticosteroids, immunosuppressive agents and
plasmapheresis or intravenous immunoglobulin.
Guillain-Barré Syndrome affects 10 to 20 million people each
year. [Australian figures 1 in 80,000-100,000] The GP must
be responsible for recognising the disease in its early stages.
Respiratory failure may develop rapidly and early hospitalisation
is essential to minimise the risk of death and other complications.
The diagnosis is essentially a clinical one depending on the history.
A high index of suspicion of the disease is necessary to prevent
tragedy, and the onus is on the GP to be alert to the possibility
of Guillain-Barré Syndrome.