Guillain–Barré syndrome

Published on 03/03/2015 by admin

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Last modified 22/04/2025

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Guillain–Barré syndrome

Definition

Guillain–Barré syndrome (GBS) is included in the wider group of peripheral polyneuropathies and is an autoimmune disease affecting the peripheral nervous system (PNS) characterized by a rapid demyelination, followed by remyelination. There are several types of GBS.

Acute inflammatory demyelinating polyneuropathy (AIDP)

This is the most common form.

Acute motor axonal neuropathy (AMAN)

Also termed acute motor and sensory axonal neuropathy (AMSAN).

Chronic inflammatory demyelinating polyneuropathy (CIDP)

As the name suggests, this involves a more chronic onset than other forms of GBS. Typically, patients with CIDP have a chronic progressive or relapsing remitting illness developing over at least 2 months but then potentially continuing for many years.

Epidemiology

Prevalence

GBS affects about one person in 40 000 each year in the UK.

Age

It can occur at any age from infancy onwards but is slightly more common in the elderly.

Gender

It is more common in men than in women.

Aetiology

The definitive cause of GBS is uncertain, however in many cases there appears to be a prior infection, e.g. glandular fever or campylobacter. Recent surgery is also recognized as a triggering event.

Pathology

GBS is thought to consist of two subtypes, demyelinating (AIDP, CIDP) and axonal forms (AMAN, AMSAN). The pathological process is related to an autoimmune response towards the nerve tissue.

Demyelination

The end result of such an autoimmune attack on the peripheral nerves is inflammation of the myelin sheath. In the peripheral nervous system, the cells that produce the myelin sheath are known as Schwann cells. Inflammatory cells invade the nerve tissue where they release cytokines which cause macrophages to attack and digest the myelin sheath.

Axonal loss

Recent studies have demonstrated that approximately 80% of patients present with demyelination, but as many as 20% also present with axonal loss. Axonal degeneration is thought to occur due to the massive disruption of sodium channels at the nodes of Ranvier (S2.6).

Outcome and prognosis

With widespread demyelination of the peripheral nerves, signal conduction (S2.6) is slowed or halted and therefore communication between systems becomes ineffective. The common course of the disease is ascending, beginning in the legs and often moving from distal to proximal body segments. Over 59% of patients reach nadir (maximum symptoms) within 2 weeks, 80% within 3 weeks and 90% within 4 weeks. A plateau period then begins, which may last 4–6 weeks but in some cases up to 1 year. The majority of patients require hospitalization and about 30% require ventilator assistance.

However, the PNS does have the ability for significant regeneration and therefore substantial functional recovery is possible. Initially Schwann cells repair the damage to the myelin sheath, however recovery dependent on axonal regeneration is much slower and there is often a greater degree of residual damage. This damage is considered to be the reason some patients are left with permanent weakness. Most of this recovery starts after the 4th week from the onset of the disease. Approximately 80% of patients have a complete recovery within a few months to a year, although minor findings may persist. About 5–10% of patients have one or more late relapses when they are classified as having CIDP. About 5–10% will be left with severe disability, with most of such cases involving severe proximal motor and sensory axonal damage. Mortality does occur in 2–3% of patients.

Signs and symptoms

The signs and symptoms presented will be a combination of:

• Secondary complications

Motor

• Muscle weakness: potentially all muscles may be affected (S3.30)

• Reflexes: Patients may become hypo-reflexic or a-reflexic (S3.22)

• Cranial nerve involvement (S2.10): Frequently, the lower cranial nerves may be affected, leading to bulbar weakness

• Dysphagia: difficulty with swallowing (CN IX and XII) (S3.16)

• Dysarthria: (CN VII, IX and XII) (S3.16)

• Facial weakness: (CN VII)

• Eye movement abnormalities: (CN III, IV and VI)

• Fatigue: This is a common and disabling symptom in patients with GBS. There are different mechanisms associated with fatigue. A generalized lack of energy most pronounced in the morning, mental exhaustion and reduced muscular endurance, which is most dramatic in the acute phase of the illness.

Sensory

• Numbness and tingling: often the initial symptoms in the extremities (S3.16,23)

• Loss of proprioception (joint position sense) (S3.23)

• Pain: common as a deep ache in weakened muscle (S3.29).

Autonomic dysfunction

• Bladder dysfunction may occur in severe cases but should be transient

• Fluctuations in blood pressure, postural hypotension and cardiac arrhythmias are possible in severe cases of GBS.

Secondary complications

• Respiratory insufficiency: Respiratory failure occurs in at least 17–30% of patients. Bulbar dysfunction, respiratory muscle weakness, and secondary insufficiency due to atelectasis or pneumonia are suggestive of its development

• Psychosocial (S3.16): Depression, anxiety, lack of confidence, change of role, financial

• Change in postural alignment (S3.20)

• Poor core stability (S3.25)

• Altered gait (S3.19)

• Poor coordination (S3.26)

• Poor balance (S3.32)

• Decreased range of movement (S3.28)

• Reduced function (S3.18)

• Falls (S3.34).

References and Further Reading

Barohn, RJ, Lewis, RA. The alphabet soup of acute and chronic immune-mediated demyelinating polyneuropathies: similarities and differences. Autumn newsletter of the GBS/CIDP Foundation International; 2005.

Food Standards Agency www.food.gov.uk/science/sciencetopics/microbiology, 2008

GBS/CIDP Foundation International www.gbs-cidp.org

Miller RG Katz, JS. Fatigue in Guillain–Barré syndrome. Summer newsletter of the GBS/CIDP Foundation International; 2005.

National Institute for Neurological Disorders and Stroke (NINDS) www.ninds.nih.gov/disorders/gbs/gbs.htm

Susuki, K, Rusband, MN. Autoantibody-mediated disruption of sodium channel clusters in peripheral motor nerve fibers in axonal Guillain–Barré syndrome. Summer newsletter of the GBS/CIDP Foundation International; 2006.

Yiu, G, Zhigang, H. Glial inhibition of CNS axon regeneration. Nature Reviews Neuroscience. 2006; 7:617–627.

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