Guillain–Barré syndrome

Published on 02/04/2015 by admin

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Last modified 22/04/2025

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94 Guillain–Barré syndrome

Instruction

Examine this patient’s lower limbs, whose weakness began distally.

Salient features

History

• Weakness: difficulty in rising up from standing position or climbing stairs, legs usually affected before upper limbs

• Dyspnoea: late in the course suggesting diaphragmatic and intercostals muscle weakness

• Cranial nerve involvement: diplopia, drooling of saliva, regurgitation of food

• Paraesthesias

• Urinary symptoms

• Systemic symptoms: fatigue

• Ascertain whether the onset was preceded by a trivial viral illness.

Examination

• Weakness of distal limb muscles

• Distal numbness

• Areflexia.

Proceed as follows:

• Tell the examiner that you would like to:

• assess respiratory function (forced vital capacity)
• check BP (for labile BP).

Remember: Polyneuropathies can be classified as demyelinating or axonal. They can also be classified according to the diameter of the affected nerve fibre. Larger fibres are heavily myelinated and, therefore, most subject to processes that damage myelin. Most polyneuropathies, including the diabetic type, are axonal.

Diagnosis

This patient has Guillain–Barré syndrome (lesion) and is currently experiencing weakness of the distal limb muscles (functional status).

Questions

What features are required for diagnosis?

• Progressive weakness in both arms and legs (might start with weakness only in the legs)

• Areflexia (or decreased tendon reflexes).

What features strongly support diagnosis?

• Progression of symptoms over days to 4 weeks

• Relative symmetry of symptoms

• Mild sensory symptoms or signs

• Cranial nerve involvement, especially bilateral weakness of facial muscles

• Autonomic dysfunction

• Pain (often present)

• High concentration of protein in CSF

• Typical electrodiagnostic features.

What feature should raise doubt about the diagnosis?

• Severe pulmonary dysfunction with limited limb weakness at onset

• Severe sensory signs with limited weakness at onset

• Bladder or bowel dysfunction at onset

• Fever at onset

• Sharp sensory level

• Slow progression with limited weakness without respiratory involvement (consider subacute inflammatory demyelinating polyneuropathy or chronic inflammatory demyelinating polyradiculoneurtopathy (CIDP)

• Marked persistent asymmetry of weakness

• Persistent bladder or bowel dysfunction

• Increased number of mononuclear cells in CSF (>50 × 106/l)

• Polymorphonuclear cells in CSF.

What is the pathology?

It is a demyelinating neuropathy. Infections (e.g. with Campylobacter jejuni) might induce an immune response that finally leads to Guillain–Barré syndrome. The immune response depends on certain bacterial factors, such as the specificity of lipooligosaccharide, and on patient-related (host) factors. Genetic polymorphisms in the patients might partially determine the severity of Guillain–Barré syndrome. Antibodies to lipooligosaccharides can cross-react with specific nerve gangliosides and can activate complement. The extent of nerve damage depends on several factors. Nerve dysfunction leads to weakness and might cause sensory disturbances (Lancet Neurol 2008; 7:939–50).

Advanced-level questions

How is the diagnosis confirmed?

• Nerve conduction studies demonstrate slowing of conduction or conduction block

• CSF shows albumino-cytological dissociation: a normal cell count but protein concentration is frequently raised.

What is Miller–Fisher syndrome?

A rare proximal variant of Guillain–Barré syndrome that initially affects the ocular muscles and in which ataxia is prominent.

What is the differential diagnosis?

Poliomyelitis, botulism, primary muscle disease or other neuropathy (porphyric, diphtheric heavy metal or organophosphorous poisoning).

How would you treat such patients?

• High-dose intravenous gammaglobulin during the acute phase to reduce the severity and duration of symptoms (N Engl J Med 1992;326:1123–9). This is equivalent to plasma exchange in effectiveness in reducing disability, but the combination of intravenous immunoglobulin and plasma exchange offers no significant additional advantage (Lancet 1997;349:225–30)

• Ventilatory support if respiratory muscles are affected

• Physiotherapy and occupational therapy for muscle weakness.

What is the prognosis?

Despite medical therapy, this syndrome often remains a severe disease: 3–10% of patients die and 20% are still unable to walk after 6 months. In addition, many patients have pain and fatigue that can persist for months or years. Outcome in patients with Guillain–Barré syndrome can be determined with the Erasmus GBS Outcome Scale (EGOS). Using EGOS, the chance of walking unaided after 6 months can be calculated on the basis of the age of the patient, the presence of diarrhoea and the severity of weakness in the first weeks. Despite treatment with intravenous immunoglobulin, many patients only partially recover and have residual weakness, pain and fatigue (Lancet Neurol 2008; 7:939–50).

What do you know about chronic inflammatory demyelinating polyradiculoneuropathy CIDP?

• This is the most common demeylinating neuropathy (N Engl J Med 2010;362:929–40).

• It is an idiopathic multifocal inflammation of the nerves that can occur at any age in the form of a subacute sensorimotor polyneuropathy.

• It is diagnosed by the findings of electrical conduction block (segmental demyelination at areas of inflammation, as seen on nerve-conduction studies) and by a high level of protein in the CSF.

• CIDP is usually idiopathic but also can occur as a feature of some connective tissue diseases.

• Corticosteroids, intravenous immunoglobulin and plasma exchange are usually effective therapies; when these are not effective, immunosuppressive drugs are often added.

• CIDP is clinically similar to Guillain–Barré syndrome except for the differing time course, and was at one time called chronic Guillain–Barré syndrome.

What do you know about POEMS syndrome?

The acronym POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) was introduced by Bardwick et al. Polyneuropathy and polyclonal plasma cell proliferation is present in all patients. In the POEMS syndrome, a clonal expansion of plasma cells occurs in association with sclerotic bone lesions (osteosclerotic myeloma). The syndrome may also involve lymph nodes in the form of angiofollicular hyperplasia, also known as Castleman disease. A moderately increased level of CSF protein in this patient is typical of the POEMS syndrome but does not distinguish it from CIDP. A low level of monoclonal IgG with lambda light chain is also characteristic of the POEMS syndrome, whereas kappa light chain predominates in monoclonal gammopathy of unknown significance (MGUS). However, in contrast to multiple myeloma, the levels of other immunoglobulin subclasses are not reduced in the POEMS syndrome.

C Guillain (1876–1961), Professor of Medicine in Paris.

JA Barré (1880–1967), Professor of Neurology in Strasbourg, trained in Paris.

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