ReviewGuillain-Barré syndrome: An update
Introduction
Guillain-Barré syndrome (GBS) is one of the acute flaccid paralysis syndromes in humans. First described in 1916 in two soldiers by French neurologists Georges Guillain, Jean-Alexandre Barré and Andre Strohl, a distinguishing feature from the then most prevalent cause of acute flaccid paralysis, poliomyelitis, was the finding of elevated cerebrospinal fluid (CSF) protein with a normal cell count, the now classic albumino–cytologic dissociation.1 Since the original description, different subtypes producing the clinical picture of GBS have been described including acute inflammatory demyelinating polyradiculoneuropathy (AIDP),2 acute motor axonal neuropathy (AMAN),3, 4 acute motor and sensory neuropathy (AMSAN),5 acute sensory neuronopathy, acute pandysautonomia and the Fisher syndrome.6
The most frequent subtype of GBS in North America and Europe is AIDP, which accounts for 90% of GBS cases, while in Asia, South and Central America, the axonal form of GBS constitutes 30% to 47% of cases.7 Only about 5% to 10% of patients in North America and Europe have an axonal subtype. Autonomic involvement is a common feature of GBS, but rarely does GBS manifest with pure autonomic dysfunction.7 In 1956, the American neurologist Charles Miller Fisher described a syndrome consisting of ataxia, areflexia and ophthalmoplegia, which has proved to be a variant of GBS. In the present review, we discuss the epidemiology, clinical features, diagnosis, pathogenesis and treatment of GBS.
Section snippets
Epidemiology
Based on well-controlled population-based studies the incidence of GBS in Europe is 1.2–1.9 cases per 100 000, while worldwide, the incidence is 0.6–4 cases per 100 000.7, 8 Atypical presentations, such as the Fisher syndrome, are much less frequent, with an incidence of 0.1 per 100 000.9 Men are 1.5 times more likely to be affected than women, and the incidence increases with age from 1 per 100 000 in those aged below 30 years, to about 4 cases per 100 000 in those older than 75 years.9 In China,
Clinical features
The dominant clinical feature of AIDP is generalised muscle weakness with sensory symptoms (excluding pain) being a relatively minor feature. In most patients, the symptoms ascend from the lower to upper limbs; however, in about one-third of cases, all limbs may be involved simultaneously and in about 10% the upper limbs may be affected first.42 Classically, both proximal and distal muscles are involved in AIDP simultaneously. Weakness of facial muscles is common, occurring in 50% of cases and
How is GBS diagnosed?
The diagnosis of GBS may be challenging and given an extensive differential diagnosis (Table 1), a thorough medical assessment of patients may be needed to exclude “mimic disorders”.7 The clinical features of ascending weakness and sensory loss, along with hyporeflexia or areflexia, should raise suspicion of GBS.7, 42, 53 Nerve conduction studies (NCS) and CSF analysis are important investigations that help confirm the diagnosis of GBS.53
Neurophysiological studies
NCS and electromyography (EMG) are important
Pathology and pathogenesis
In the AIDP form of GBS, pathological studies reveal patchy multifocal mononuclear cell infiltrates throughout the peripheral nervous system, with the distribution of inflammation often corresponding to the pattern of clinical deficit.66, 67 Activated macrophages invade intact myelin sheaths resulting in myelin damage and demyelination.67 The immunological mechanisms underlying the macrophage-mediated invasion of nerves remain elusive, although two potentially complementary mechanisms have been
Prognosis
Although GBS reaches a nadir at 2 weeks to 4 weeks, with most patients recovering from this debilitating illness, 10% to 20% of patients are left with disabling motor deficits and 4% to 15% of patients die by 1 year after onset. Up to one-third of GBS patients need to make substantial changes in their job, hobbies or social activities due to the residual functional deficit.126, 127 Adverse prognostic factors include older age at disease onset (>50 years); severe disease at nadir as indicated by
General treatment of GBS
Treatment of GBS patients requires a multidisciplinary approach.7, 131 General supportive treatment includes monitoring and controlling pulse rate and blood pressure because 5% to 61% of GBS patients may suffer wide fluctuations in blood pressures and cardiac arrhythmias due to autonomic involvement.42 Vital capacity should be monitored because about 25% of GBS patients require artificial ventilation, which should be considered once the vital capacity falls below 15 mL/kg to 20 mL/kg. In
Plasmapheresis and intravenous immunoglobulin (IVIg)
Plasmapheresis has been the accepted as the gold standard of treatment for GBS for almost 20 years. In four trials that included 585 participants, plasmapheresis resulted in significant improvement in clinical features within 4 weeks of randomisation, as indicated by an improvement in disability and increased proportion of patients recovering to full strength within 1 year.133, 134, 135, 136 In a further five trials with 623 participants, plasmapheresis reduced the percentage of patients
Conclusions
Although the pathophysiological mechanisms and efficacy of different immunomodulatory treatments have been studied extensively over the last 20 years, about 20% of GBS patients are left with a functional disability and 60% report severe fatigue at 12 months.7, 157, 158 Further research is required in understanding the underlying pathophysiology of different GBS subtypes that may result in development of novel therapies. For example, if the T cells were of prime importance in AIDP, then agents
Acknowledgements
Funding support from the Motor Neuron Disease Research Institute of Australia (MNDRIA) and National Health and Medical Research Council of Australia is gratefully acknowledged.
References (163)
- et al.
Clinical and electrophysiological aspects of acute paralytic disease of children and young adults in northern China
Lancet
(1991) - et al.
Guillain-Barré syndrome
Lancet
(2005) - et al.
Guillain-Barré syndrome after vaccination in United States a report from the CDC/FDA Vaccine Adverse Event Reporting System
Vaccine
(2007) - et al.
Guillain-Barré syndrome following facial bone fracture
J Plast Reconstr Aesthet Surg
(2006) - et al.
Guillain-Barré syndrome following coronary artery bypass surgery
Am Heart J
(1987) - et al.
Guillain-Barré syndrome following cardiopulmonary bypass
Int J Cardiol
(1992) - et al.
Guillain-Barré syndrome secondary to cranial surgery: direct or fortuitous relationship?
Neurochirurgie
(2005) - et al.
Miller Fisher syndrome with transient coma: comparison with Bickerstaff brainstem encephalitis
Brain Dev
(2002) - et al.
Neurophysiologic findings in early acute inflammatory demyelinating polyradiculoneuropathy
Clin Neurophysiol
(2004) - et al.
Reduced circulating CD4+CD25+ cell populations in Guillain-Barré syndrome
J Neuroimmunol
(2007)
Temporal profile of anti-ganglioside antibodies and their relation to clinical parameters and treatment in Guillain-Barré syndrome
J Neurol Sci
From ionic currents to molecular mechanisms: the structure and function of voltage-gated sodium channels
Neuron
Excitability of human axons
Clin Neurophysiol
Conduction slowing without conduction block of compound muscle and nerve action potentials due to sodium channel block
J Neurol Sci
Sur un syndrome de radiculonevrite avec hyperalbuminose du liquide cephalo-rachidien sans reaction cellilaire. Remarques sur les caracteres cliniques et graphiques des reflexes tendineux
Bull Soc Med Hop Paris
Acute idiopathic polyneuritis. An electron microscope study
Lab Invest
Acute motor axonal neuropathy: a frequent cause of acute flaccid paralysis in China
Ann Neurol
Pathology of the motor-sensory axonal Guillain-Barré syndrome
Ann Neurol
An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia)
N Engl J Med
Clinical and epidemiologic features of Guillain-Barré syndrome
J Infect Dis
Incidence and clinical features of acute inflammatory polyradiculoneuropathy in Lombardy, Italy, 1996
Acta Neurol Scand
Distinct pattern of age-specific incidence of Guillain-Barré syndrome in Harbin, China
J Neurol
Guillain-Barré syndrome: epidemiology, pathophysiology and management
Drugs
Guillain-Barré syndrome following vaccination in the National Influenza Immunization Program, United States, 1976–1977
Am J Epidemiol
An epidemiologic and clinical evaluation of Guillain-Barré Syndrome reported in association with the administration of swine influenza vaccines
Am J Epidemiol
Guillain-Barré syndrome and its relationship to swine influenza vaccination in Michigan, 1976-1977
Am J Epidemiol
Guillain-Barré Syndrome in recipients of A/New Jersey influenza vaccine
JAMA
Guillain-Barré syndrome and the 1978–1979 influenza vaccine
N Engl J Med
Guillain-Barré syndrome in the United States, 1979–1980 and 1980–1981: lack of an association with influenza vaccination
JAMA
Guillain-Barré syndrome and influenza vaccination in the US Army, 1980–1988
Am J Epidemiol
Guillain-Barré syndrome following influenza vaccination
JAMA
No association between immunization and Guillain-Barré syndrome in the United Kingdom, 1992 to 2000
Arch Intern Med
The Guillain-Barré syndrome and the 1992–1993 and 1993–1994 influenza vaccines
N Engl J Med
Guillain-Barré syndrome after influenza vaccination in adults: a population-based study
Arch Intern Med
Guillain-Barré syndrome following acute head trauma
Postgrad Med J
Guillain-Barré syndrome and head trauma. Case report
Arq Neuropsiquiatr
Guillain-Barré syndrome after mandibular surgery: report of case
J Oral Surg
Cardiorespiratory arrest as a complication of Guillain-Barré syndrome in a postsurgical patient
J Oral Surg
Guillain-Barré syndrome after heart transplantation
J Heart Lung Transplant
An unusual nursing challenge: Guillain-Barré syndrome following cranial surgery
J Neurosci Nurs
Guillain-Barré syndrome following oesophagectomy
ANZ J Surg
Cell-mediated immunity in severely head-injured patients: the role of suppressor lymphocytes and serum factors
J Neurosurg
Stress and immunity in humans: a meta-analytic review
Psychosom Med
Psychological stress and disease
JAMA
Elevated serum titers of proinflammatory cytokines and CNS autoantibodies in patients with chronic spinal cord injury
J Neurotrauma
Guillain-Barré syndrome
Neurol Sci
Recurrent Guillain-Barré Syndrome: a clinical, electrophysiological and morphological study
J Assoc Physicians India
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