Guillain-Barré Syndrome

Dpt. Aamir Memon

26.08.2013

Guillain-Barré Syndrome (acute idiopathic polyneuropathy)

Definition: Guillain-Barré syndrome (GBS) is a condition involving acute inflammation and

destruction of the myelin layer of peripheral nerves.

It usually starts in the extremities and moves toward the

trunk, but some variants of this syndrome affect only cranial

nerves or have other patterns.

→ Guillain-Barré syndrome is a peripheral nervous system disease characterized

by the sudden onset of muscle paralysis or paresis.

→ Guillain-Barré results from an autoimmune attack against the myelin

surrounding the peripheral nerves. With destruction of the myelin, the axons

can be damaged.

→ Symptoms of Guillain-Barré disappear as the autoimmune attack ceases and the

axons regenerate. If destruction of the cell body occurred during the attack,

some degree of disability may remain.

→ Although the cause of Guillain-Barré is unknown, the disease usually occurs 1

to 4 weeks after a viral infection or immunization.

The muscles of the lower extremities are usually affected first,

with paralysis advancing up the body.

Respiratory muscles may be affected, leading to respiratory

collapse.

Cardiovascular function may be impaired because of interruption

of autonomic nerve function.

It is an inflammatory, demyelinating disease whose etiology is not completely

understood but probably immunologic in origin.

It affects people of any age, sex, or race and is characterized by extreme

weakness and numbness or tingling in the extremities and a loss of movement

or feeling in the upper body and face progressing to paralysis.

There is an association with infections, vaccinations, and surgery.

Most clients have good recovery but it may take months.

GBS was first described in 1916, but our understanding of how this disease

comes about has not progressed a great deal since then.

Many patients have an infection of the respiratory or gastrointestinal tract

several days before developing GBS symptoms.

It is believed that this preceding infection stimulates an immune system attack

mistakenly directed against the myelin sheaths of peripheral nerves.

Demographics: → GBS can affect anyone any time, but most affected people are 15-35 or 50-75

years old.

→ Men with GBS slightly outnumber women.

→ Although it is not a particularly common problem, affecting about 1 in every

100,000 people (about 3,000 people in the United States each year), it is the

most frequently seen form of acute neuromuscular paralysis since the

eradication of polio in the Western Hemisphere.

Etiology:

→ Research indicates that a common cause of food poisoning, the bacterium

Campylobacter jejuni, may trigger many cases of GBS in the United States.

Other pathogens that have been linked to GBS include Haemophilus

influenzae, Mycoplasma pneumoniae, Borrelia burgdorferi, cytomegalovirus,

Epstein-Barr virus, and HIV.

→ Some GBS patients don’t experience a preceding infection. This disorder has

also been seen in conjunction with immune system changes brought about by

pregnancy, surgery, and administration of certain vaccines, specifically the

swine flu vaccine that was distributed in 1976.

→ Regardless of what initiates the disease process, the end result is that the

myelin sheaths on peripheral nerves are attacked and destroyed by

macrophages and lymphocytes.

The damage progresses proximally and may also affect cranial

nerves.

This can be life threatening if the nerves that control breathing

are damaged; many GBS patients spend time on a ventilator

before they recover.

→ GBS is now recognized as several different subtypes of demyelinating diseases.

The most common form in the United States is acute inflammatory

demyelinating polyneuropathy; this accounts for 90% of GBS diagnoses. Other

types include these:

Acute motor axonal neuropathy affects motor neurons only.

It is most common in children and has a good prognosis.

Acute motor-sensory axonal neuropathy affects motor and

sensory function.

It is most common in adults and has a poorer recovery rate

than other forms of GBS.

Miller-Fisher syndrome is a rare variant of GBS that involves

only the cranial nerves.

It leads to poor control of the eyes and other facial muscles.

→ The cause of Guillain–Barré syndrome is unknown, but in about 50% of cases, the onset

follows the infections:

Causes Why Viral infection, bacterial infection,

common cold, mononucleosis, hepatitis,

gastrointestinal (GI) infection,

Pathogens in these infections, such as C. jejuni in

GI infection, are thought to alter the immune

system, causing T-lymphocytes to be sensitized

inoculations to myelin and to trigger demyelination

Signs and Symptoms → GBS is notorious for being unpredictable, but it has a few features that

distinguish it from other peripheral nerve disorders.

→ Onset is typically fast and severe; a patient may go from being fully functional

to being hospitalized within a matter of hours or a couple of days.

→ GBS is usually symmetrical, affecting both legs equally.

→ Also, myelin damage progresses proximally, moving up toward the trunk rather

than distally; this pattern is unique among peripheral nerve problems.

→ When GBS first appears, it often involves weakness or tingling in the affected

limbs.

→ Reflexes become dull or disappear altogether.

→ Loss of sensation progresses proximally, although pain frequently develops in

the hips and pelvis.

→ If the GBS affects cranial nerves of the face, facial weakness, pain, and

difficulty with speech and swallowing may develop.

→ As the disease progresses, the nerves that supply respiratory muscles are

affected, and problems with breathing develop.

→ GBS symptoms usually peak 2 or 3 weeks after onset, and they may linger for

several weeks before they begin to subside.

→ The amount of damage that accrues while the nerves are inflamed depends on

what treatments were introduced when and on how soon the patient can begin

to use the affected muscles after the paralysis resolves.

→ Weakness usually begins in the legs, then spreads to involve the arms and face.

→ Respiratory muscles may be involved.

→ Life-threatening complications can occur such as tachycardia, arrhythmias, and

pulmonary dysfunction

The signs and symptoms and rationales associated with Guillain–Barré syndrome:

Signs and symptoms Why Numbness Nerve impulses slow down or cease

Tingling in fingers or toes Nerve impulses slow down or cease

Mild difficulty in walking With denervation, muscles atrophy

Complete paralysis of the extremities With denervation, muscles atrophy

Diagnosis Guillain–Barré syndrome is difficult to diagnose because of the varied symptoms, but

if symptoms occur uniformly across the body and progress rapidly, the diagnosis is

made much easier.

→ Lumbar puncture may be performed to look for elevated proteins in

cerebrospinal fluid which is common.

→ Nerve conduction test may be recommended to confirm that nerve transmission

to the extremities is impaired.

Treatment

→ Because GBS is an idiopathic disease, no specific cure has been developed.

→ Two treatment options have been successfully used to shorten recovery time:

1. Plasmapheresis (blood cleansing).

This removes autoimmune antibodies and reduces attacks against

myelin.

This procedure is most effective within 2 weeks of onset.

2. Injections of high concentrations of IV immunoglobulin (donated antibodies).

Inhibit the patient’s antibody and cytokine activity, thus limiting the

autoimmune attack against myelin sheaths on peripheral nerves.

These options can shorten the recovery process by up to 50%.

→ About one-third of patients require the use of a ventilator until the respiratory

nerves regain full function.

→ Anticoagulants may be used against the danger of blood clots in immobilized

legs.

→ Pain management is problematic because powerful pain medications can

depress the nervous system; massage and other nondrug options are often

recommended for this purpose.

→ Once the acute inflammation has passed, occupational and physical therapy is

used to help the patient regain as much muscle function as possible.

MODALITY RECOMMENDATIONS FOR GBS

MODALITY RECOMMENDATION

Deep Tissue Massage

Indicated while subacute. Work in areas with full sensation to

improve proprioception. Encourage active movement with release

work during a long recovery.

Lymphatic drainage Supportive.

Polarity S: Indicated.

R/D: Locally contraindicated while acute; otherwise indicated.

PNF/MET/stretching Supportive.

Reflexology Indicated; all glands, brain, head, spine, solar plexus points.

Shiatsu

Indicated. Treat immune system via TH/SP/K; nervous system

via BL, SI.

Swedish massage Contraindicated while acute; otherwise indicated to help restore

function. Caution for areas of reduced sensation.

Trigger point therapy Locally contraindicated while acute; indicated when subacute

with caution for areas of reduced sensation.

Prognosis

→ The good news is most people who develop GBS have a full or nearly full

recovery, although the process may take 18 months or longer.

→ Many people live with permanent loss of some neurological function (foot drop

or numbness in an area, for instance), but these are not considered disabling

problems.

→ A small number of patients (5%–15%) have permanent serious disability as a

result of the disease.

→ About 10% of GBS patients have a relapse later in life.

→ About 5% to 7% of GBS patients die, usually of respiratory failure, pulmonary

embolism, or cardiac arrest.

Complications

→ Respiratory or cardiovascular collapse may cause death.

→ Weakness of some muscles may persist.