Guillain-Barré Syndrome – Symptoms, Causes and Treatment

What is Guillain-Barré Syndrome?

Guillain–Barré Syndrome (GBS) is an inflammatory disease that involves the degeneration of the myelin sheath of the peripheral nerves. GBS is also termed as Guillain–Barré –Strohl syndrome, Landry’s paralysis, acute idiopathic polyradiculoneuritis, French polio, or cute idiopathic polyneuritis. It is considered an acute polyneuropathy. Guillain–Barré syndrome affects people of all ages and races.

Guillain-Barré Syndrome Showing Demyelination of the Peripheral Nerve

The characteristic feature of Guillain–Barré syndrome is ascending weakness and paralysis, beginning in the lower extremities and spreads rapidly to the trunk, upper extremities and face in a period of four weeks. It becomes a life-threatening disorder when the paralysis already involves the respiratory muscles, which may lead to respiratory arrest.

Guillain–Barré syndrome is usually preceded by an infection and may develop after one to four weeks of infection.

Mechanism of Guillain-Barré Syndrome

Guillain–Barré syndrome is caused by previous infection. The antigens attack the peripheral nervous system which stimulates the body to produce antibodies against it. The resulting infection in the peripheral nerves produce an inflammatory process in the area. Antibodies mistakenly attack the peripheral nerves eventually damaging the myelin sheath.

The myelin sheath in the nerves is the fatty insulating layer responsible for nerve impulse conduction. Due to its absence, nerve impulse transmission is affected leading to impairment in muscle contraction and subsequently paralysis. Absence of myelin sheath also affects the autonomic regulation.

History of Guillain–Barré Syndrome

Jean Landry in 1859 first described the disorder. However, the term Guillain–Barré syndrome was taken from three physicians who discovered the presence of elevated protein in the spinal fluid of two soldiers diagnosed with GBS. These physicians were Guillain, George, Barré, Jean Alexandre, and Strohl, Andre.

Guillain-Barré Syndrome Symptoms

Signs and Symptoms of Guillain–Barré syndrome include:

1. Symmetrical weakness starting from the lower limbs
2. Paresthesia
3. Loss of deep tendon reflexes
4. Deep, aching muscle pain in the shoulder girdle and thighs
5. Ascending weakness affecting the face, upper extremities and trunk over a period of four weeks, but may develop rapidly in hours or days
6. Facial weakness
7. Dysphagia (difficulty of swallowing when muscles of the throat are affected)
8. Drooling
9. Shortness of Breath
10. Difficulty of breathing (when respiratory muscles are affected)

Autonomic Dysfunction may also occur when the autonomic nervous system is already affected as manifested by:

1. orthostatic hypotension
2. hypertension
3. papillary disturbances
4. sweating dysfunction
5. cardiac dysrhythmias
6. paralytic ileus
7. urinary retention

Guillain-Barré Syndrome Causes

Most of the cases of Guillain–Barré syndrome have unknown etiology. However, causes are often associated with infections. Most common infections that develop into Guillain–Barré syndrome are:

Campylobacter jejuni infection

Campylobacter jejuni is usually present in cases of food poisoning.

Cytomegalovirus

Cytomegalovirus (CMV) is a genus of herpesviridae virus responsible for several infections such as pneumonia, hepatitis, gastritis, colitis and neurologic infections. CMV is usually found in the saliva of certain mammals including cats.

Influenza Virus

Some cases are triggered by influenza virus. However, some cases of influenza virus immunization during the swine flu pandemic in the 1976 produced a post immunization reaction that led to Guillain–Barré syndrome. Present influenza virus vaccines do not lead to such condition.

Types of Guillain-Barré Syndrome

There are six types of Guillain–Barré syndrome. These include:

Miller Fisher Syndrome

Miller Fisher Syndrome is a rare type of Guillain–Barré syndrome, which is responsible for about 5% of all cases. Paralysis in MFS occurs in an opposite manner, which is descending paralysis.

Acute inflammatory demyelinating polyneuropathy

This type of GBS is the most usual type of GBS affecting the Schwann cells.

Acute Motor Sensory Axonal Neuropathy

This type results in severe axonal damage and affects the sensory nerves.

Acute Motor Axonal Neuropathy

This is also known as the Chinese paralytic syndrome. It attacks the nodes of Ranvier in the neuron. This type is seasonal and there is rapid recovery. It is common in Mexico and China.

Acute panautonomic neuropathy

This is the rarest type of GBS. It is considered the most severe form because it leads to encephalopathy and cardiac dysrhythmias. It has a high mortality rate and poor prognosis.

Bickerstaff’s brainstem encephalitis

It is an additional type of GBS, which is of remitting-relapsing type. This type involves presence of inactive and active periods of disease.

Guillain-Barré Syndrome Diagnosis

Primary diagnosis of Guillain–Barré syndrome includes presence of symptoms such as descending paralysis, and areflexia (absence deep tendon reflex) preceded by a previous infection. Diagnosis of Guillain–Barré syndrome involves several tests to come up with a definite diagnosis. These include:

Electrodiagnostics

Nerve conduction study (NCS) and electromyelography (EMG) is involved. These tests examine the electrical activity within the neurons. EMG and NCS reveal slowed conduction in nerves that are demyelinated. In areas of axonal damage, there is decrease in action potentials.

Cerebrospinal Fluid Analysis3

The CSF of a patient with Guillain–Barré syndrome has elevated protein level as a result of the inflammatory response. CSF is collected through lumbar tap with the use of a thin spinal needle. Once the needle is in place, CSF is aspirated and subjected for analysis.

The diagnostic criteria of Guillain–Barré syndrome involve presence of progressive weakness on the extremities, areflexia, and less than four weeks course of the disease along with other symptoms.

Gullain-Barré Syndrome Treatment

The acute phase of the disease requires rapid medical measures to avoid severe complications. Supportive management is the main treatment of Guillain–Barré syndrome because of respiratory involvement. Specific treatments include:

Ventilator Assistance

Intubation is required with patients who suffer from respiratory paralysis. The muscles for respiration does not contract leading to inability to expand and create a negative pressure inside the thorax. Tracheostomy, the creation of an opening into the trachea from the anterior neck, is also employed when intubation is not possible.

Plasmaparesis

This involves the removal of circulating antibodies through filtering the blood. The removal of antibodies that damage the myelin sheath prevents further demyelinization and injury. Plasmaparesis needs to be instituted within four weeks of the first signs and symptoms appearing.

Intravenous Immunoglobulins

Immunoglobulins are administered to counteract the harmful ones that attack the peripheral nerves. Plasmaparesis and immunoglobulin administration is essential for reducing the circulating harmful antibodies. Intravenous immunoglobulin should be administered within two weeks of the signs and symptoms appearing.

Glucocorticoids

Administration of glucocorticoids such as dexamethasone reduces the inflammatory reaction on the neurons. However, glucocorticoids have not been found to be equally effective with immunoglobulin administration and plasmaparesis.

After these treatments, rehabilitation is needed to return the patient to optimum level of functioning and enable the patient to perform activities of daily living. Physical therapists assisting in improving the strength of the limbs and improving the gait. Speech therapists are also needed to help the patient to regain the speaking and swallowing ability.

Complications

Complications of GBS include:

1. Bed sores
2. Contractures of muscles
3. Cardiac arrhythmias
4. Calcium stones (as a result of immobility)
5. Respiratory Arrest

Prognosis

About 90% of patient recover from GBS within four weeks from the time signs and symptoms appear. Most of the cases have full recovery and only a small percentage end up with complications and permanent damage. Mortality rate of Guillain–Barré syndrome has remained to be about 3% despite advancement in technology and drugs.

There is a good prognosis with most of the cases. Poor prognosis occurs in patients who are 40 years old and above, patients who require ventilator support, have a history of diarrheal disease preceding the GBS and those who have poor muscle strength on the upper extremities because this indicates respiratory affectation.

Life Expectancy

Guillain–Barré syndrome is a life-threatening disease that needs immediate attention in order to prevent further ascending of paralysis. When treatment is started early, complications are reduced and prognosis is good. Remissions also do not affect the life expectancy of patients.

Prevention

Prevention of Guillain–Barré syndrome requires avoidance of causative factors such as infection. Preventing Campylobacter jejuni infection requires limiting intake of processed foods and checking for the expiry date. Prevention of influenza is also crucial to reduce incidence of Guillain–Barré syndrome. Influenza vaccines are available and needed every year especially during cold seasons.

References:

• http://www.mayoclinic.com/health/guillain-barre-syndrome/DS00413
• http://emedicine.medscape.com/article/315632-overview
• http://www.betterhealth.vic.gov.au/bhcv2/bhcarticles.nsf/pages/Guillain-Barre_syndrome
• http://www.bbc.co.uk/health/physical_health/conditions/guillainbarre1.shtml