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GBS Guillain- Barré Syndrome Lauren Nowak Saginaw Valley State University 1 GBS 2 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 GBS 3 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 GBS 4 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 GBS 5 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). GBS 6 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 GBS 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 7 GBS 8 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. GBS 9 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).