Vol. 21 Issue 12
It comes on fast but usually retreats significantly
Guillain-Barre Syndrome (GBS) is an acute inflammatory disorder that affects the peripheral nerves, spinal nerve roots, and occasionally selected cranial nerves. This condition is often referred to as acute idiopathic neuropathy, infectious polyneuritis and Landry's syndrome." GBS progresses rapidly resulting in muscular weakness and mild sensory loss.
While the condition typically affects the legs, weakness and abnormal sensations can extend to the arms and upper body. These patients are often placed on ventilators due to weakened respiratory muscles and abnormal blood pressure and heart rate.
The underlying etiology is still unknown; many times GBS follows a recent surgical procedure, viral infection or immunizations. GBS equally affects both sexes and can develop at any age; however, it is most common between the ages of 30 and 50. It is a rare syndrome, afflicting only about one person in 100,000.
It is known that GBS is not an inherited syndrome; however, there are speculations that link genetics to developing certain autoimmune conditions as a result of an acute infection.
While the cause of GBS is unknown, there are many theories related to associated risk factors. Many times, the condition occurs due to antecedent viral infections such as mononucleosis or acquired immunodeficiency disorder. In addition, GBS can occur after a recent surgery, receiving vaccines for rabies or swine flu, and lastly as a result of systemic lupus erythematosus or Hodgkin's disease.
Clinical Signs and Symptoms
The onset of GBS varies from hours to days, and often emerges over three to four weeks. GBS is often referred to as ascending paralysis because it begins in the legs and progresses to the arms.
The following symptoms are typical at the onset of GBS: muscle weakness, sensation changes, blurred vision, difficulty moving face muscles, palpitations, muscle contractions, difficulty swallowing, drooling, difficulty breathing, syncope, weakened muscles of inspiration/expiration, clumsiness and falling.
The signs and symptoms are often an important diagnostic tool for GBS. Physicians find it difficult to diagnose GBS in the earliest stages because the etiology is not clear. However there are a few tests performed to aid in making a diagnosis. It is common for Guillain-Barre patients to present with symptoms bilaterally, and reflexes such as knee jerks are lost; therefore, it is important for physicians to make note of this.
Typically, symptoms appear in days or weeks that can aid in ruling out other disorders which progress much slower. Usually, the transmissions of signals along nerve fibers are slower (demyelination) in GBS; therefore a nerve conduction velocity may be performed.
Physicians may choose to perform a spinal tap to determine if cerebrospinal fluid contains an elevated level of protein without an increase in white blood cell count, common in GBS patients. In addition, an electromyography (EMG) test is can determine if muscle activity shows decreased stimulation. An electrocardiograph (ECG) may indicate abnormalities in a number of cases.
Magnetic resonance imaging (MRI) or computed tomography (CT) scanning can rule out other diseases. Pulmonary function tests help to measure neuromuscular respiratory function, diaphragmatic strength and determine if respiratory assistance is warranted.
A tracheotomy may be needed to address respiratory failure, particularly in patients on a mechanical ventilator for two weeks or more.
Currently there are no known cures; however, certain treatments can reduce the severity of the syndrome, increase recovery time and thwart complications related to GBS. Typically, GBS patients with difficulty breathing and other body functions are placed in the intensive care unit (ICU) for help with respiration and monitoring.
Plasmapheresis, a blood cleansing procedure that removes plasma that may be potentially toxic to the myelin, can often reduce the severity and duration of a Guillain-Barre episode. Scientists are unsure why this procedure is successful in treating GBS patients. High-dose immunoglobulin therapy also reduces the severity and duration of GBS. Specifically, this treatment weakens the immune system's attack on the nervous system (NINDS, 2001).
In the past, steroid hormones were used to do this, but in multiple clinical trials, studies have shown that these drugs may have a more harmful effect on the disease process.
Preventive treatments include blood thinners to decrease incidence of blood clots; dietary changes; use of feeding tubes or positioning to reduce choking hazards; intermittent bladder catheterization to avoid urinary tract infections, urinary retention or sepsis; and anti-inflammatory agents and narcotics to control pain.
Psychological counseling may be necessary to assist patients with adjustment to paralysis and dependency in numerous occupations. Caregivers often are trained to perform passive range of motion (PROM) to maintain muscle strength and flexibility and decrease incidence of contractures.
GBS can be divided into three phases: the initial phase, plateau phase and the recovery phase.
The initial phase occurs after the first symptom emerges; it ends in days or weeks. In the plateau phase symptoms stabilize and can last several days to a few weeks. The recovery phase varies, lasting only a few weeks or as long as years.
It is thought that the recovery phase is a period when remyelination and axonal process regrowth occur.
Nearly 95 percent of all patients survive GBS; however, a number of patients never recover completely. Typically, the prognosis improves if symptoms do not persist beyond three weeks of the initial onset. About three percent may suffer a relapse of muscle weakness and tingling sensations many years after the initial attack.
Due to the sudden onset of the disease, the patient may become fearful that he or she will become dependent on someone for daily tasks. Due to the degree of uncertainty regarding recovery and the ability to care for themselves, Guillain-Barre patients may express feelings of frustration and helplessness. As a result of these frustrations, tolerance for social interaction may be limited in the early stages of recovery.
Role of OT
The focus of treatment is primarily remedial, while compensatory techniques should be considered to allow performance of some activities in the early stages. An individual's strength and endurance are important factors when formulating a proper treatment plan.
If the OT is considering the use of assistive technology, he or she should use it only on a short-term basis to avoid dependency.
When addressing the patient's self-care, the OT should begin treatment with sedentary activities that do not further compromise strength and endurance. Splinting may be useful for protection against contractures and/or skin breakdown. Arm supports or slings may allow increased performance of daily activities such as eating. Temporary modifications in the home and workplace may be necessary to allow the person to engage in activities of daily living (ADL).
Leisure interests might be incorporated into the treatment regimen. Taking this avenue may help to motivate the individual to participate in the treatment session.
A number of sensorimotor components should be addressed, including:
• maintenance of PROM, AROM;
• proper positioning, using splints to prevent deformity and contractures; and
• graded activities to increase muscle strength and endurance, decrease joint stiffness, and increase fine-motor coordination and dexterity.
Discuss joint protection principles with the patient and those individuals involved with treatment implementation, and incorporate sensory stimulation and neuromuscular re-education.
Teaching stress management and relaxation techniques are helpful in addressing anxiety associated with the disease process.
Finally, provide positive feedback and encouragement; try to lessen the patient's anxiety about potential dependency.
Currently, the focus of research is dedicated to treatment of GBS, both existing and new methods. As stated, GBS may occur after a viral or bacterial infection; therefore, scientists are searching for answers as to why the immune system malfunctions.
In addition, studies are being conducted to determine exactly which cells are responsible for this destruction of the nervous system. According to the National Institute of Neurological Disorders and Stroke (NINDS), (2001), "certain proteins or peptides in viruses and bacteria may be the same as those found in myelin, and the generation of antibodies to neutralize the invading viruses or bacteria could trigger the attack on the myelin sheath."
References available at www.advanceweb.com/OT or upon request.
Tracy Morris, COTA/L, works at Lankenau Hospital in Havertown, PA, in the transitional care center. In addition, she is in her final year of the weekend OTR program at College Misericordia. She can be reached at firstname.lastname@example.org.