Guillain-Barré syndrome (GBS) is an acute inflammatory disorder of the peripheral nervous system that results in muscle weakness and paralysis. It is caused by an autoimmune attack against the myelin sheath surrounding nerves. Symptoms include numbness and tingling in the extremities that progresses up the body. While most patients recover fully, treatment such as plasmapheresis or immunoglobulin therapy can help shorten recovery time from several months to weeks. GBS has several subtypes and is usually preceded by a viral or bacterial infection. Prognosis is generally good, though some permanent weakness may remain in a small percentage of cases.
2. 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.
3. 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
4. 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.
5. 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.
6. 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.