Guillain-Barre Syndrome

Abstract and Introduction

Abstract

Guillain-Barré syndrome is a heterogeneous condition with several variant forms that is the most common cause of acute flaccid paralysis in both children and adults. It is estimated that the condition affects about 1 to 3 individuals per 100,000 persons. Patients typically complain of increasing pain and weakness in the limbs associated with tingling dysesthesias in the extremities. The majority of patients with Guillain-Barré have been diagnosed with an infection in the 3 weeks prior to the development of symptoms. Other events that have been linked to the disorder include vaccinations, surgery, and certain drugs. Therapy includes immunotherapy to accelerate the recovery process as well as specific therapies for individual patients such as pain relief.

Introduction

Guillain-Barré syndrome (GBS) is a heterogeneous condition associated with immunemediated, reactive, self-limiting peripheral neuropathies.^[1,2] It represents at least five different entities that are associated with an increased concentration of cerebrospinal fluid protein but a normal cell count and manifest as systemic motor paralysis.^[1,2]

Three of these forms predominantly affect the motor system: acute motor axonal neuropathy (AMAN), acute motor-sensory axonal neuropathy (AMSAN), and acute inflammatory demyelinating polyradiculoneuropathy (AIDP).^[1] The other two variants are Miller Fisher syndrome and acute pandysautonomic neuropathy.^[1]

GBS is the leading cause of flaccid paralysis in Western countries. It is estimated that direct healthcare costs of patients in the United States alone amount to $110,000 per person, and costs due to a loss in productivity per patient run close to $360,000 annually.^[2,3]

The disorder, triggered by a preceding bacterial or viral infection, causes respiratory failure requiring mechanical ventilation in approximately 25% of cases.^[2] Mortality rates in patients requiring mechanical ventilation are as high as 20%, with persistent disability and persistent fatigue in 20% and 67% of patients, respectively.^[4]

Table 1. Antecedent Events in GBS
Event	Examples
HLA predisposing pattern	Genetic predisposition
Immunoglobulin KM genes	Genetic predisposition
Vaccines	Rabies and swine influenza
Viral infections	Cytomegalovirus, Epstein-Barr virus, varicella zoster virus
Bacterial infections	Campylobacter jejuni, mycoplasma pneumonia
Protozoan infections and infestation	Chlamydia pneumoniae, acute toxoplasmosis
Surgery	Mandible surgery, operation of the spine, cardiac surgery
Blood transfusion and transplantation	Related to risk of infection
Drugs	Antibiotics, allopurinol, thiabendazole, danazol, corticosteroids, D-penicillamine, gold salt, vincristine, zimelidine, stavudine, organophosphate poisoning, arsenic poisoning

GBS: Guillain-Barré syndrome; HLA: human leukocyte antigen.
Source: References 2, 10.

Table 2. Features of GBS
Type	Symptoms	Pathology
AIDP	Most common variant (85% of cases); primarily motor inflammatory demyelination ± secondary axonal damage; max of 4 wk of progression	Macrophages invade intact myelin sheaths and denude the axons
AMAN	Motor only with early and severe respiratory involvement; primary axonal degeneration; often affects children, young adults; up to 75% positive Campylobacter jejuni serology; often positive for anti-GM1, anti-GD1a antibodies	Macrophages invade the nodes of Ranvier where they insert between the axon and the surrounding Schwann-cell axolemma, leaving the myelin sheath intact
AMSAN	Motor and sensory involvement with severe course of respiratory and bulbar involvement; primary axonal degeneration with poorer prognosis	Similar to AMAN but also involving ventral and dorsal roots
Miller Fisher syndrome	Ophthalmoplegia, sensory ataxia, areflexia; 5% of all cases; 96% positive for anti-GQ1b antibodies	Abnormality in sensory conduction, although the underlying pathology is not clear
Acute Pandysautonomic neuropathy	Most rare form; may be accompanied by encephalopathy	Widespread sympathetic and parasympathetic failure

AIDP: acute inflammatory demyelinating polyradiculoneuropathy; AMAN: acute motor axonal neuropathy; AMSAN: acute motor-sensory axonal neuropathy; GBS: Guillain-Barré syndrome; max: maximum. Source: References 17–20.

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Guillain-Barré Syndrome

Abstract and Introduction

Abstract

Introduction

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Tables

References

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