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A GP's Guide to Guillain Barré Syndrome & CIDP

By James G McLeod AO, FRCP (Lond), FRACP

Reprinted from Modern Medicine of Australia October 1995

DEFINITION

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 nerve.
(a) top. Normal myelinated fibre with nerve impulse being conducted from
node to node, causing muscle contraction.
(b) bottom. Autoimmune attack on myelin causes a block in nerve conduction,
preventing muscle contraction. Plasmapheresis and intravenous immunoglobulin
inhibit immune-mediated attack on myelin, allowing remyelination to commence
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 Syndrome.

PATHOGENESIS
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.

DIAGNOSIS
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

• Transverse myelitis
• Myasthenia gravis
• Poliomyelitis
• Acquired hypokalaemia
• Periodic paralysis
• Polymyositis
• Botulism

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
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

• HIV infections
• Acute intermittent porphyria
• Vitamin B deficiencies
• Heavy metals and other toxins
• Lymphoma, carcinoma
• Lyme disease
• Vasculitis
• Diptheria
• Diabetes

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.

SPECIFIC THERAPY

Plasmapheresis
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 drugs
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.
COUNSELLING
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.

INVESTIGATIONS
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 infection.
Table 4 - Management of Guillain-Barré Syndrome
• Admit to hospital • Monitor vital capacity • Treat respiratory failure:
• Specific therapy:
• Intragastric or intravenous feeding may be necessary
• General nursing care:
• Prevent deep venous thrombosis:
• Physical therapy

• 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 weakness. • Bilateral facial weakness should alert the clinician to the diagnosis. • 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 of complications. • Twenty per cent of patients will have residual physical disability requiring long-term management and GP supervision at home.

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.

SUMMARY
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.