Download Guillain-Barré Syndrome

GBS
Guillain- Barré Syndrome
Lauren Nowak
Saginaw Valley State University
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Introduction
Guillian Barre Syndrome, or GBS, is a type of neuromuscular paralysis that can affect
anyone regardless of sex, race, or age. Most commonly it affects males slightly more than
females during the age range of 50 to 74. However, there is contradictory evidence proving that
this disease also affects 16 to 25 year olds. A diagnosis is made by ruling out other neurological
conditions and obtaining a patient’s history. Within 48 hours total paralysis and cardiac arrest
can result. This disease progresses rapidly and can even result in death if no treatment is
received, although, the central nervous system, cerebral function, and consciousness is not
normally affected. This condition affects two out of 100,000 people each year. What makes this
disease fascinating to health professionals is its unknown cause (Toft, 2002).
Etiology
The cause of GBS is not completely understood. One factor believed to play a role in the
onset of this disease is an infection preceding GBS within 1 to 3 weeks. Almost two-thirds of
patients have reported a respiratory tract or gastrointestinal infection prior to the onset of GBS.
The most prevalent infection is Campylobacter jejuni, which accounts for 26 percent of this
population. Within two months after contracting Campylobacter jejuni the risk for developing
GBS increases 100 times. Other diseases that are linked to GBS are: Mycoplasma pneumonia,
Cytomegalovirus, Haemophilus influnzae, Eshericia coli, Epstein Barr, and human
immunodeficiency virus (Khan, 2009).
Other factors believed to be related to GBS are acute illness, trauma, surgery,
immunizations, Herpes simplex, and Mononucleosis. In 1976, the swine flu vaccination was
thought to have cause rare cases of GBS, but today the flu vaccines have not resulted in any
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cases of GBS. These factors are believed to sensitize the T-cells to the patient’s own myelin.
This process creates a toxic effect to the nerves (Kahn, 2009).
There are three theories that explain the viral link to GBS. First, the illness creates an
autoimmune response that affects the T- cell suppressor circuits. Secondly, the body attacks itself
because the viral antigen has comparable surface markers as the myelin. Lastly, the cranial and
spinal nerves are affected in this process (Toft, 2002).
Pathophysiology
GBS can be called other names such as acute idiopathic polyneuritis and
polyradioculoneuropathy. It is a result of immune mediated pathological responses. The myelin
sheath of the axons of the peripheral nervous system is attacked and possibly the axon itself is
attacked by the immune system. The main pathologic finding in GBS is segmental
demyelination, or the damage of myelin between the nodes of Ranvier (Sulton, 2002).
When demyelination occurs, the impulse cannot travel as quickly from node to node. In
addition, the brain cannot receive as many sensory signals. This will affect the patient’s ability to
feel heat, textures, and pain. In some cases, the brain can receive altered signals in which the
patient feels a “crawling sensation.” Cerebrospinal fluid protein levels are usually elevated in the
second week of the illness (Kahn, 2004).
When examined under a microscope, there were large groups of lymphocytes found at
sites of myelin breakdown, although, the axons remained intact. In some rare cases the cell body,
neurolemma, or axon are damaged. This can cause lasting neurological deficits (Sulton, 2002).
Clinical Course
During the acute course of GBS there are three stages. In the acute or initial period,
which usually last one to four weeks, begins with the onset of symptoms and ends when there is
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no more progression of the disease. The plateau period usually last several days to two weeks.
Then finally there is the recovery period, which can last four to six months or up to six years.
During this phase remyelination and axon regeneration occurs. However, some patients do not
completely recover and have lasting neurological deficits (Sulton, 2002).
Symptomaology
There are four variants of GBS: Ascending GBS, Pure Motor GBS, Descending GBS,
and Muller Fisher Variant. Ascending GBS is the most common clinical pattern. The symptoms
in this variant include weakness, paresthesias, starts with leg pain in lower extremities and
progresses to the trunk and arms, affect on the cranial nerves, ascending flaccidity that evolves or
hours to days, mild paresis to total quadriplegia, repiratory compromise, and absent deep tendon
reflexes in paralyzed muscles. In the Pure Motor GBS variant they symptoms are similar to
ascending GBS, but the patient is in less pain and sensory manifestations are not present (Toft,
2002).
In the descending GBS the onset of symptoms starts with weakness of the face, neck,
jaw, tongue, pharynx, and larynex muscles. The weakness then progresses downward to the
upper extremities, trunk, and lower extremities. Usually respiratory system is affected causing
shallow breathing, breathlessness during speech, dyspnea, and decreased tidal volume. There is
also ophthalmoplegia or weakness of eye muscles resulting in diplopia. If the pupils are affected
functional blindness can result. Deep tendon responses are absent and numbness usually occurs
more frequently in the hands compared to feet (Toft, 2002).
The last type of GBS is the Muller Fisher variant, a rare polyneuropathy. This cause
ophthalmoplegia, areflexia, and ataxia. In this type motor strength and sensory function are not
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affected, but on occasion papillary response to light is disturbed and can cause functional
blindness (Toft, 2002).
Prognosis
The prognosis for GBS is a favorable outcome for most patients. Most people survive the
disease and recover completely. The recovery process usually starts two to four weeks after the
onset. The entire course of GBS lasts for about twelve weeks. Fifty percent of patients have
minor residual neurological deficits, and fifteen percent have persistent residual deficits in
function. About eighty percent are ambulatory within six months of the disease onset (Khan,
2004).
There are some factors involved that can be used to predict a poorer outcome such as:
older age, need for respiratory support, rapid onset, progression to quadriplegia, severe disease
presentation, C. jejuni infection, and preceding diarrheal illness. Some of the neurophysiological
characteristics associated with a poorer outcome are: summed motor velocity less than eighty
percent of normal and summed proximal and distal motor amplitudes of less than twenty percent
of normal. There is now a scoring scale for predicted function recovery for GBS. It takes in
consideration of a person’s age, preceding diarrhea, and the GBS disability score at two weeks. It
is used to accurately predict the outcome of ambulation at six months (Khan, 2009).
OT Role and General Treatment
The advancement in technology and medical care has improved the survival rates of
GBS, and now there is a reduced mortality rate of less than five percent. Common complications
that can arise in GBS are: sepsis, aspiration, pneumonia, deep vein thrombosis, pulmonary
embolism, cardiac arrest, and respiratory failure (Sulton, 2002).
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A common treatment for GBS is plasmapheresis, which eliminates the traveling
antibodies that are responsible for the disease. This is done usually within several days of onset
to up to thirty days. The patient receives treatment three to four times, one to two days apart.
This treatment is used to shorten the amount of time a patient remains on a respirator, shortens
the time to achieve independent walking, and can help reach functional mobility at the six month
mark (Sulton, 2002).
In the past steroids were used in the treatment of GBS to decrease the severity of the
illness. Newer research has found that using methylpredisolone is ineffective in decreasing the
severity. Another treatment that is occasionally used is immunoglobulin infusion. This treatment
has been proven to be just as effective as plasmapheresis, safer, and more readily available.
Some of the side effects of immunoglobulin infusion are: chills, fever, myalgia, headache,
aseptic meningitis, retinal necrosis, and acute renal failure (Hughes, 2005).
Forty percent of all GBS cases require inpatient rehabilitation. Many patients are disabled
and may need a ventilator in the acute phase of this condition. Patients must be monitored for
respiratory distress, and may require intubation. They are then transferred back into the hospital
for medical stabilization. A typical case of GBS would require the patient to stay in inpatient
rehabilitation for three to six weeks, followed by outpatient rehabilitation for three to four
months. During this time the goal is to maintain functional independence as soon as the patient is
medically stable and using a team of occupational therapist, physical therapist, nurses, and social
workers (Kahn, 2004).
Occupational therapy for neurological rehabilitations designs a program to increase
function, reduce limitations, and enhance the overall well-being of patients who have suffered
from a disorder of the nervous system. The main goal is help the patient achieve the highest level
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of function in the activities of daily living. In an outpatient setting, Occupational Therapists will
perform musculoskeletal and neurological assessments to determine baseline functions and
cognitive status (Foster, 2004).
Then the therapist will design a treatment plan with specific interventions to reach
goals. Some activities an Occupational Therapist will work with patients on are: functional
mobility, coordination, balance, increasing range of motion, retraining in activities of daily
living, activities to improve cognitive functioning, measure safety issues, and
recommendations for assistive devices. Occupational Therapists may also make splints to help
manage flaccidity caused from GBS (Guillain barre syndrome, 2001).
Community Support Agencies
Although most people recover from GBS, there are a few who do not fully recover. For
those who are fortunate to have a full recovery, the patients and their family go through an
emotional trauma, not knowing what outcome to expect. The Gullian- Barre Syndrome
Foundation International provides patients and their families support with educational
literature, visits from patients who have experienced the disease previously, develops national
support groups, provides resources for patients with long term disabilities, and donates funds
to research for finding a cure and alternative treatment options. There are two chapters of the
Guillain- Barre Syndrome Foundation International in Michigan located in Ann Arbor and
Portage. For more information on the Foundation or for the local chapters go to www.gbscidp.org ("Gbs/cidp foundation international," 1997).
Conclusion
GBS is a condition with rapid onset that can impact ones functional capacity. Its exact
cause is unknown, but a gastrointestinal or respiratory infection prior to the onset of GBS is
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reported in about 60 percent of all GBS cases. During this disease the body mistakes the myelin
sheath and destroys it. GBS has three stages in its course: acute, plateau, and recovery. The
prognosis for a fully functional recovery is very good. The occupational therapist’s role in the
treatment of this condition is to regain function, reduce limitations, and enhance the patients
overall well-being. Lastly, there are support groups out there for the patient, families, and friends
of people with lasting effects of Guillian Barre Syndrome.
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References
Foster, E.C. (2004). Muscle belly tenderness, functional mobility, and length of hospital stay in
the acute rehabilitation of individuals with guillain barre syndrome. Journal of
Neurologic Physical therapy, 28(4)
Gbs/cidp foundation international. (1997). Retrieved from http://www.gbs-cidp.org/
Guillain barre syndrome. (2001). Unpublished manuscript, Mayo Clinic, Rodchester, Minnesota.
Retrieved from http://www.mayoclinic.org/guillain-barre-syndrome/
Hughes, R.(2005, November). Guillian- Barre Syndrome. The Lancet.
Kahn, F. (2004). Rehabilitation in guillian barre syndrome. Australian Family Physician. 33(12).
Kahn, F. (2009). Guillian- Barre Syndrome: An update in rehabilitation.
International Journal of Therapy and Rehabilitation, 16(8).
Sulton, L.L. (2002, July). Meeting the challenge of guillian barre syndrome. Nurisng
Management.
Toft, C.E. (2002). Guillian-barré syndrome – a case study. Accident and Emergency Nursing,
10(2).