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Guillain-Barre SyndromeDacy GastonSouth University, SavannahGuillain-Barre SyndromeGuillain-Barre syndrome (GBS) is a rare post-infective autoimmune disorder that causes demyelination of the body’s peripheral nerves. Most cases of GBS are curable and self-limiting following rapid recognition of the contributing symptoms and prompt treatment of the disease. The annual occurrence of GBS is approximately 1 to 2 per 100,000 or around 3500 cases per year, which makes this a unique, and often times misdiagnosed syndrome (Woodward, 2013). It is imperative that the healthcare professional complete a full history and physical on the patient to determine the diagnosis of GBS. This disease affects every aspect of the patient’s life because of its rapid degradation of the peripheral nervous system that can lead to full body paralysis. Hospitalization is often required with supportive therapy and interdisciplinary intervention for full recovery. This literature review aims to explain the full extent of GBS by addressing the following elements:PathophysiologyClinical Presentation, Symptoms, and Disease ProgressionDiagnostic and TestingClinical Management and TreatmentConcepts to Consider in Treating Guillain-Barre SyndromeCare Plan/Case Study of Guillain-Barre SyndromeUnderstanding the unique characteristics that define GBS will allow the healthcare provider to better recognize, diagnose, and treat the disease promptly. The prognosis of the patient relies heavily on the correct diagnosis by the healthcare provider. With rapid discovery and treatment many patients can and will fully recover. PathophysiologyGuillain-Barre syndrome is a rare disease in which the immune system attacks the gangliosides on the peripheral nervous system (PNS). The understanding of the pathophysiology of GBS has greatly improved over the past decade from its early detection in 1916 by three doctors named Jean-Alexandre Barré, Georges Guillain, and Andre Strohl, who first discovered the disease in soldiers during World War I (Pluta, Lynm, & Golub, 2011). Originally thought, GBS was a progressive motor disorder associated with absent reflexes and raised protein in cerebrospinal fluid. Today, GBS is categorized in three different forms varying with the level of severity. The first, acute inflammatory demyelinating polyradiculoneruopathy (AIDP), is the most frequent type and is primarily the demyelinating of the axonal nerves. The second, acute motor axonal neuropathy (AMAN), is the next most frequent and affects just the motor nerves. The third, acute motor and sensory axonal neuropathy (AMSAN), is the rarest and involves both the sensory and motor nerves (Winer, 2014, p.1). Most GBS cases are preceded by a respiratory or gastrointestinal infection a few days or a few weeks before onset of symptoms. Two-thirds of all cases of GBS are preceded by Campylobacter jejuni infection. Other microbial hosts that have thought to be associated with GBS include Epstein-Barr virus, Mycoplasma pneumonia, and cytomegalovirus. Recently following the swine flu virus vaccine in 1976, an increase in the incidence of GBS cases caused the correlation of viral immunizations to be another precursor in developing GBS (Walberg, 2013). After the trigger of microbial or viral infection, the body’s immune system responds by activating macrophages to invade the myelin sheaths resulting in the peripheral nerve demyelination. To this day the exact immunological response mediated by the macrophages is unknown. In the AIDP form of GBS, it is thought that activated CD4 (T cells) react against specific antigens on the surface of the Schwann cells in the PNS, and therefore directly activating the macrophages to this area (Vucic, Kiernan, & Cornblath, 2009, p. 735, para. 3). This antibody activation leads to a destruction in the PNS which causes muscle weakness and sometimes paralysis. In concurrence of the T cell pathogenesis in GBS, the importance of antibodies against the gangliosides whose lipid portion is located in the cell membrane also plays a part in the pathogenesis of the disease. Antibodies against specific gangiosides that reside on the nervous system have been reported in cases of GBS. The anti-GM1 antibodies have been found in the patients that have the AMAN form of GBS. The role of the anti-GM1 antibodies cause dysfunction of voltage-gated sodium channels, resulting in failure of conduction, which leads to muscle weakness (Vucic, et al., 2009, p. 735, para. 6). Regardless of the pathological course, GBS progresses into bilateral ascending paralysis with the loss of reflexes and often times in severe cases it can cause loss of respiratory function.Clinical Presentations, Symptoms, and Disease ProgressionGBS has three distinct phases; the acute, the plateau, and the recovery phase. Symptoms begin usually in the acute phase that can last from a few days to a few weeks. In the plateau phase, symptoms often “plateau” and last anywhere from a few days to a few weeks. The recovery phase is after symptoms have regressed and improvement in health is noted. The extent of disease lingering in the three phases depends on treatment and recognition to slow and regress symptoms (Lugg, 2010).GBS often mimics other diseases such as spinal cord compression, poliomyelitis, stroke, hypokalemia, myasthenia gravis, Lyme disease and diabetes mellitus (Vucic et al., 2009). Because of the rarity of GBS diagnosis, the healthcare professional must do a proper comprehensive health physical to rule out other diagnoses. According to Walling & Dickson (2013), the most common clinical presentations before the onset of GBS are fever, sore throat, cough, and malaise. As the disease progresses further, symptoms typically include bilateral muscle weakness, pain, and numbness and tingling in the extremities. Respiratory failure can occur in rapid progression of the disease if the muscle weakness and paralysis reaches the respiratory muscles. Facial and oculomotor muscles may be affected, but are found in the more rare forms of GBS (pp. 192-193). Pain is also a hallmark symptom in 50 to 89% of patients with GBS and mimics sciatica symptoms. In most cases of GBS the symptoms are extremely broad, and prompt treatment of these patients is crucial because the window of time in the more advanced cases of GBS can mean life or death in patients where the respiratory muscles are involved (Walling & Dickson, 2013, p. 193). The disease progression and prognosis of GBS is generally good as long as proper detection and correction is accomplished. From initial onset of symptoms most cases of GBS last anywhere from two to six weeks, with varying levels of intervention depending on severity of the individual case. Most patient require physical therapy after treatment that can last for months. Factors that influence disease progression and prognosis include age, degree of severity, and pre-GBS infection determinant. Increased age and rapid severe onset of GBS has a higher adverse prognosis. Infection with Campylobacter jejuni or cytomegalovirus can cause a longer recovery period depending on infection reason (Vucic et al., p. 737). Diagnostic and TestingIn the case of expected GBS, the patient requires a multidisciplinary team of healthcare professionals to determine to diagnosis of GBS. Diagnosis of GBS relies on recognizing the symptoms associated with peripheral nerve demyelination, and to differentiate it from other disease that mimic muscle weakness. The criteria for diagnosis includes a comprehensive neurological exam, specific testing of the cerebrospinal fluid (CSF), specific blood tests, systematic rule out diagnosis, and electro diagnostic testing such as nerve conduction tests (McGrogan, Madle, Seaman, & de Vires, 2009).According to Walling and Dickson (2013), evaluation required to diagnose GBS is based on comprehensive physical and neurological tests. Suspected GBS usually begins in the legs and is bilateral in ascending nature. Diminished neurological reflexes and acute extremity weakness peaks within two weeks from onset of symptoms. Clinical diagnosis supportive of GBS includes autonomic, sensory, and cranial involvement. Cardiac arrhythmias, facial weakness, and sensory diminishment ensue as the disease progresses. Lumbar puncture to collect cerebrospinal fluid will show elevated protein levels, with a normal white cell count. Blood test rule out diagnosis of symptoms of disease that can mimic GBS such as B-12 deficiency, diabetes mellitus, hypokalemia, infection, and malignancy (p. 192). When diagnosing GBS, neurophysiology is extremely useful (Winer, 2014). Because GBS is difficult to diagnose early on in the progression, neurophysiology can assist in early diagnosis. Early assessment findings by neurophysiology shows small action wave potential, prolonged distal motor latency, delayed F waves (nerve conduction velocity test), and delayed conduction block (p. 3). Initial abnormalities are found in 85% of GBS cases in the early phase of the disease and can help aid in prompt correct diagnosis (Pritchard, 2010). Diagnosis is then determined when all the subjective and objective data is collected and compared to confirm GBS. Clinical Management and TreatmentWhen a patient is diagnosed with GBS, interdisciplinary management provides a comprehensive approach to patient care and management. Healthcare professionals include nurses, Neurology doctors, infectious disease doctors and rheumatologist. They are all part of the interdisciplinary team during diagnosis. Patients diagnosed with GBS can become fully paralyzed, proper education by the interdisciplinary team is vital for the mental health of the patient. Knowing what is to come can aid in making the patient more calm and understanding the disease progression. Physical treatment includes general supportive therapy at aims of improving muscle strength, controlling bowel and bladder function, monitoring and controlling blood pressure, pulse, respiratory function, and cardiac abnormalities (Walling & Dickson, 2013). Paralysis is a cornerstone symptom in GBS, hospitalized patients are at high risk for deep venous thrombosis (DVT) because of their immobile state. Vucic et al. (2009) explain that DVT prophylaxis, anticoagulant and antiplatelet treatment, and compression stockings is beneficial (p. 737, para. 6). A multidisciplinary rehabilitation program is also imperative to recovery. Patients can benefit from inputs from a physiotherapist and occupational therapist, along with joining a support group for GBS survivors (p. 737, para 7). Intravenous immune globulin therapy and plasmapheresis has been shown to speed up recovery time in GBS patients. Plasmapheresis was the accepted gold standard for treatment in GBS until the induction of intravenous immune globulin therapy (Vucic et al., 2009, p.738). According to Walling & Dickson (2009), “intravenous immune globulin therapy, given at 400 mg per kg per day for five days, has significantly fewer complications than plasma exchange” (p. 195, para. 4). Early evidence introduced the treatment of GBS with corticosteroids, but has since been erased from treatment regimen (p. 195, para. 6). Concepts to Consider in Treating Guillain-Barre SyndromeGBS is a costly disease. Most patient require hospitalization for days and sometimes up to weeks and months. Hospital stays are a huge financial burden to the economy and the patient. Supportive therapy for the patient includes the labor of the doctors, nurses, and other interdisciplinary teams involved. Often times even after the patient recovers and goes home they are followed up by occupational health, or physical rehabilitation. Added to the hospital stay, costs of treatment come from the infusion of therapeutic plasma exchange (TPE) and/or intravenous immunoglobin (IVIg) therapy. According to Winters et al. (2011), “the average cost of five treatments of inpatient IVIg is approximately $10,325.00, and the average cost of TPE is approximately $4,600.00” (pp. 2-3). Because of the costs associated with GBS, patients of a lower socioeconomic status or without healthcare insurance may have difficulty in being able to pay for these treatments. Patient and family members are directly affected by this disease. GBS can cause full body paralysis, which can lead to ventilator assisted breathing. Bernsen et al. (2010) states that psychological distress on patient and family members was prevalent in the intensive care unit with patients diagnosed with GBS. Anxiety, fear, forced dependency, inability to communicate, pain, and lack of information all contributed to the patient and family member’s psychological distress (p. 537).Healthcare professionals have a responsibility to the patient and family members to provide as much information as possible about the treatment and disease process of GBS. Understanding and open communication can lessen the psychological stress for the patient and family. Care Plan/Case Study of Guillain-Barre SyndromeComprehensive ExamDemographic: 28 year old Caucasian FemaleChief Complaint: “I’m having numbness and tingling in both of my feet that seems to have gotten worse over the past two days accompanied by feeling tired and weak.”HPI: 28 yr old Caucasian female presents to clinic with bilateral numbness and tingling in both her lower extremities which has gotten worse over the last few days. Nothing makes it better. Sleeping 10 to 12 hours per night, and having a hard time getting out of bed in the morning from fatigue. Stomach flu two weeks ago in which she had diarrhea and nausea with loss of appetite. She is currently eating small amounts and is adequately hydrated. Current Medications:Oral Birth Control – Orthotrycycline 1 po q dayMultivitamins – one “Women’s multivitamin” q dayNo Drug Allergies or seasonal allergiesPast Medical History Fully immunized, Giardasil, Hep B.Past Surgical HistoryGallbladder removal 2006Social HistorySingle Non smokerDrinks two-three glasses wine per weekFamily HistoryFather-HTNMother-type 2 Diabetes diagnosed at 45Paternal Grandfather- HTN, Type 2 DiabetesPaternal Grandmother- Breast CancerMaternal Grandfather- CHFMaternal Grandmother-Type 1 DiabetesReview of SystemsGeneral: No fever, night sweats, lymphadenopathySkin: no lesions, sores, rashHEENT: No headaches or visual disturbances. No eye tenderness or redness. No decreased hearing or tinnitus. No nasal discharge. No dysphagia.Neck: No stiffness or massesPulmonary: No cough or shortness of breathCardiovascular: No angina, palpitations or edema.Gastrointestinal: No vomiting, constipation, or melena.Genitourinary: No frequency of urination, burning or pain.Musculoskeletal: No joint swelling, soreness or stiffness.Neurological: No headaches, seizures or gait problems.Endocrine: No excessive thirst, weight gain or polyuria. Physical AssessmentVital signs: 110/78, HR 68, Temp 99.9, RR 18, Height 5’8, Weight 128lbs, BMI 19.4General: Patient is alert, oriented has apathy and fatigue.HEENT: Eyes: Pupils equal round and reactive to light. No visual disturbances.Ears: No erythema, discharge or effusionNose: Nares swollen, red.Throat: Uvula visualized, redness and swelling of tonsils.Neck: Thyroid not palpable, no massChest: Lung: Breath sounds clear bilaterally, no rales, wheezes.Heart: Regular rhythm. Normal S1, S2, no murmur.Abdomen: Bowel sounds present, No masses. Extremities: Numbness and paresthesia in lower extremities. Skin warm to touch, skin turgor normal.Neurological: Sensory diminished in lower extremities with diminished deep tendon lower extremity reflexes. Differential/Diagnosis:Metabolic DisturbancesGuillain-Barre syndromePlan:CBC with differentialCMPFinger stick Referral to Neurology ASAP for EMGFollow up with Neurology considering strong evidenced of Guillain-Barre syndrome. References:Bernsen, R., de Jager, A., Kuijer, W., van der Meche, F., Suurmeijer, T. (2010). Psychological dysfunction in the first year after Guillain-Barre Syndrome. Muscle & Nerve, 41, 533-539.Lugg, J. (2010). Recognizing and managing Guillain-Barre syndrome. Emergency Nurse, 8(3), 27-30.McGrogan, A., Madle, G., Seaman, H., de Vries, C. (2009). The epidemiology of Guillain-Barre syndrome worldwide. Neuroepidemiology, 32, 150-163.Pluta, R., Lynm, C., Golub, R. (2011). Guillain-Barre Syndrome. JAMA, 305(3), 319.Pritchard, J. (2010). Guillain-Barre syndrome. Clinical Medicine, 10(4), 399-401.Vucic, S., Kiernan, M., Cornblath, D. (2009). Guillain-Barre syndrome: an update. Journal of Clinical Neuroscience, 16, 733-741.Walberg, M. (2013). The risk of Guillain-Barre syndrome after influenza vaccination. Formulary, 48, 303.Walling, A., Dickson, G. (2013). Guillain-Barre syndrome. American Family Physician, 87(3), 191-197.Winer, J. (2014). An update in Guillain-Barre syndrome. Autoimmune Diseases, 2014, 1-6.Winters, J., Brown, D., Hazard, E., Chainani, A., Andrzejewski, C. (2011). Cost-minimization analysis of the direct costs of TPE and IVIg in the treatment of Guillain-Barre syndrome. BMC Health Services Research, 11, 1-8.Woodward, S. (2013). Guillain-Barre syndrome. British Journal of Neuroscience Nursing, 9(2), 59-61. ................
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