29/11/13

Guillain-Barre Syndrome Treatment & Management

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Guillain-Barre Syndrome Treatment & Management

Author: Michael T Andary, MD, MS; Chief Editor: Robert H Meier III, MD more... Updated: Aug 29, 2012

Approach Considerations
Patients who are diagnosed with GBS should be admitted to a hospital for close monitoring until it has been determined that the course of the disease has reached a plateau or undergone reversal. Although the weakness may initially be mild and nondisabling, symptoms can progress rapidly over just a few days. Continued progression may result in a neuromuscular emergency with profound paralysis, respiratory insufficiency, and/or autonomic dysfunction with cardiovascular complications. Approximately one third of patients require admission to an intensive care unit (ICU), primarily because of respiratory failure. After medical stabilization, patients can be treated on a general medical/neurologic floor, but continued vigilance remains important in preventing respiratory, cardiovascular, and other medical complications. Patients with persistent functional impairments may need to be transferred to an inpatient rehabilitation unit. Continued care also is needed to minimize problems related to immobility, neurogenic bowel and bladder, and pain. Early involvement of allied health staff is recommended. Early recognition and treatment of GBS also may be important in the long-term prognosis, especially in the patient with poor clinical prognostic signs, such as older age, a rapidly progressing course, and antecedent diarrhea.[86] Immunomodulatory treatment has been used to hasten recovery. Intravenous immunoglobulin and plasma exchange have proved equally effective.
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Good supportive care is critical in the treatment of patients with GBS.[87] Because most deaths related to GBS are associated with complications of ventilatory failure and autonomic dysfunction, many patients with GBS need to be monitored closely in ICUs by physicians experienced in acute neuromuscular paralysis and its accompanying complications. Competent intensive care includes the following features: Respiratory therapy Cardiac monitoring Safe nutritional supplementation Monitoring for infectious complications (eg, pneumonia, urinary tract infections, septicemia) Approximately one third of patients with GBS require ventilatory support. Monitoring for respiratory failure, bulbar weakness, and difficulties with swallowing help to anticipate complications. Proper positioning of the patient to optimize lung expansion and secretion management for airway clearance is required to minimize respiratory complications. Serial assessment of ventilatory status is needed, including measurements of vital capacity and pulse oximetric monitoring. Respiratory assistance should be considered when the expiratory vital capacity decreases to less than 18 mL/kg or when a decrease in oxygen saturation is noted (arterial PO2 < 70 mm Hg). Tracheotomy may be required in a patient with prolonged respiratory failure, especially if mechanical ventilation is required for more than 2 weeks. Close monitoring of heart rate, blood pressure, and cardiac arrhythmias allows early detection of life-threatening situations. Critically ill patients require continuous telemetry and close medical supervision in an ICU setting. Antihypertensives and vasoactive drugs should be used with caution in patients with autonomic instability. Hemodynamic changes related to autonomic dysfunction are usually transitory, and patients rarely require longterm medications to treat blood pressure or cardiac problems.
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Enteral or parenteral feedings are required for patients on mechanical ventilation to ensure that adequate caloric needs are met when the metabolic demand is high. Even patients who are off the ventilator may require nutritional support if dysphagia is severe. Precautions against dysphagia and dietary manipulations should be used to prevent aspiration and subsequent pneumonias in patients at risk. Prevention of secondary complications of immobility is also required. Subcutaneous unfractionated or low molecular weight heparin and thromboguards are often used in the treatment of immobile patients to prevent lowerextremity deep venous thrombosis (DVT) and consequent pulmonary embolism (PE). Prevention of pressure sores and contractures entails careful positioning, frequent postural changes, and daily range-of-motion (ROM) exercises. Pain management with analgesics and adjunct medications also may be needed. Modalities such as transcutaneous electrical nerve stimulation (TENS) and heat may prove beneficial in the management of myalgia. Desensitization techniques can be used to improve the patient's tolerance for activities. Although bowel and bladder dysfunction is generally transitory, management of these functions is needed to prevent other complications. Initial management should be directed toward safe evacuation and the prevention of overdistention. Monitoring for secondary infections, such as urinary tract infection, also is an area of concern. Nephropathy has been reported in pediatric patients.[88] Hospitalized patients with GBS may experience mental status changes, including hallucinations, delusions, vivid dreams, and sleep abnormalities.[89] These occurrences are thought to be associated with autonomic dysfunction and are more frequent in patients with severe symptoms. Such problems resolve as the patient recovers. Psychiatric problems such as depression and anxiety may also occur.

Physical Therapy
Estimates suggest that approximately 40% of patients who are hospitalized with GBS require inpatient rehabilitation. Unfortunately, no long-term rehabilitation outcome studies have been conducted, and treatment is often based on experiences with other neurologic conditions. The goals of the therapy programs are to reduce functional deficits and to target impairments and disabilities resulting from GBS. Early in the acute phase of GBS, patients may not be able to fully participate in an active therapy program. At that stage, patients benefit from daily ROM exercises and proper positioning to prevent muscle shortening and joint contractures. Addressing upright tolerance and endurance also may be a significant issue during the early part of rehabilitation. Active muscle strengthening can then be slowly introduced and may include isometric, isotonic, isokinetic, or progressive resistive exercises. Mobility skills, such as bed mobility, transfers, and ambulation, are targeted functions. Patients should be monitored for hemodynamic instability and cardiac arrhythmias, especially upon initiation of the rehabilitation program. The intensity of the exercise program also should be monitored, because overworking the muscles may, paradoxically, lead to increased weakness. In a study by Gupta et al in 35 patients (27 with classic GBS and 8 with acute motor axonal neuropathy), GBSrelated deficits included neuropathic pain requiring medication therapy (28 patients), foot drop necessitating anklefoot orthosis (AFO) use (21 patients), and locomotion difficulties requiring assistive devices (30 patients). At 1-year follow-up, the authors found continued foot drop in 12 of the AFO patients. However, significant overall functional recovery had occurred within the general cohort.[90]

Occupational and Recreational Therapy

Occupational therapy professionals should be involved early in the rehabilitation program to promote upper body strengthening, ROM, and activities that aid functional self-care. Both restorative and compensatory strategies can be used to promote functional improvements. Energy conservation techniques and work simplification also may be helpful, especially if the patient demonstrates poor strength and endurance. Participation in recreational therapy assists in the patient's adjustment to disability and improves integration into the community. Recreational activities, either new or adapted, can be used to promote the growth, development, and independence of a long-term hospital patient.

Speech Therapy
Speech therapy is aimed at promoting speech and safe swallowing skills for patients who have significant oropharyngeal weakness with resultant dysphagia and dysarthria. In ventilator-dependent patients, alternative communication strategies also may need to be implemented. Once weaned from the ventilator, patients with tracheostomies can learn voicing strategies and can eventually be
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weaned from the tracheostomy tube. Cognitive screening also can be performed conjointly with neuropsychology to assess for deficits, since cognitive problems have been reported in some patients with GBS, especially those who have had an extended ICU stay.

Immunotherapy
Plasma exchange, carried out over a 10-d period, may aid in removing autoantibodies, immune complexes, and cytotoxic constituents from serum and has been shown to decrease recovery time by 50%. A review of 6 randomized controlled trials involving 649 participants found that plasma exchange helps speed recovery from GBS without causing harm apart from being followed by a slightly increased risk of relapse.[91] In well-controlled clinical trials, the efficacy of IVIGs in GBS patients has been shown to equal that of plasma exchange.[92, 93, 94, 95] IVIG treatment is easier to implement and potentially safer than plasma exchange, and the use of IVIGs versus plasma exchange may be a choice of availability and convenience. Immunotherapy for children with GBS has not been rigorously studied with randomized, well-controlled studies, but is a standard aspect of treatment in this age group.[96] Immunotherapy for pregnant women has not been studied, and safety for use during pregnancy has not been established. Corticosteroids (oral or IV) have not been found to have a clinical benefit.[97] Consequently, this class of drugs is not currently employed in GBS treatment. A few studies have investigated other medications to treat GBS; however, the trials have been small and the evidence weak,[98] highlighting the need for further investigation of potential treatment options.

Analgesia
Pain medications may be required in both the inpatient and outpatient settings. A tiered pharmacologic approach that starts with nonsteroidal anti-inflammatory drugs or acetaminophen, with narcotic agents added as needed, is usually recommended. Most patients do not require narcotic analgesics after the first couple of months of illness. Adjunct medications for pain, such as tricyclic antidepressants and certain anticonvulsants, also may be beneficial for dysesthetic-type pains.

Consultations
Consultation with a neurologist can be helpful in the initial diagnosis, workup, and treatment of patients admitted to the medical floor with GBS. Critical care specialists may be required for patients in the ICU to help manage respiratory failure and multiple medical complications. Consultation with a pulmonologist may be needed to perform workup and to manage respiratory issues, such as acute respiratory distress syndrome (ARDS), pneumonia, and respiratory failure. Consultation with a cardiologist may be required if significant cardiovascular complications, such as labile blood pressure and cardiac arrhythmias, arise from the associated autonomic dysfunction. Consultation with a surgeon may be required for the placement of tracheostomies, enteral feeding tubes, and central lines. Physical medicine and rehabilitation specialists should evaluate patients for impairments and disabilities arising from the disease and should help to determine the most appropriate setting for and intensity of rehabilitation care.

Long-Term Monitoring
Although follow-up studies generally have assessed patients 6-12 months after onset, some studies have reported continued improvements in strength even beyond 2 years. With prolonged recovery possible, GBS patients with continued neurologic deficits may benefit from ongoing physical therapy and conditioning programs. Numerous papers have addressed the issue of persistent fatigue after recovery from GBS.[87, 99, 100] Studies have suggested that a large percentage of patients continue to have fatigue-related problems, subsequently limiting their function at home, work, and during leisure activities. Treatment suggestions range from gentle exercise to improvement in sleep patterns to relief of pain or depression, if present. A study of amantadine found it no more effective than placebo for relief of fatigue in GBS patients.[101] GBS can produce long-lasting changes in the psychosocial status of patients and their families.[73, 74, 75] Changes
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in work and leisure activities can be observed in just over one third of these patients, and psychosocial functional health status can be impaired even years after the GBS event. Interestingly, psychosocial performance does not seem to correlate with the severity of residual physical function. Poor conditioning and easy fatigability may be contributory factors. Therefore, providing long-term attention and support for this population group is important.

Contributor Information and Disclosures

Author Michael T Andary, MD, MS Professor, Residency Program Director, Department of Physical Medicine and Rehabilitation, Michigan State University College of Osteopathic Medicine Michael T Andary, MD, MS is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, American Medical Association, and Association of Academic Physiatrists Disclosure: Allergan Honoraria Speaking and teaching Coauthor(s) Joyce L Oleszek, MD Assistant Professor, Department of Physical Medicine and Rehabilitation, University of Colorado at Denver Health Sciences Center, The Children's Hospital of Denver Joyce L Oleszek, MD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation Disclosure: Nothing to disclose. Angela Cha-Kim, MD Assistant Professor, Director of Spinal Cord Injury, Department of Physical Medicine and Rehabilitation, Loma Linda University Medical Center Angela Cha-Kim, MD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation and American Paraplegia Society Disclosure: Nothing to disclose. Chief Editor Robert H Meier III, MD Director, Amputee Services of America; Active Medical Staff, Presbyterian/St Luke's Hospital, Spalding Rehabilitation Hospital, Select Specialty Hospital; Consulting Staff, Kindred Hospital Robert H Meier III, MD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation and Association of Academic Physiatrists Disclosure: Nothing to disclose. Additional Contributors Heather Rachel Davids, MD Physician, Department of Anesthesiology, Interventional Pain Medicine, University of Colorado Health Sciences Center Heather Rachel Davids, MD is a member of the following medical societies: American Academy of Pain Medicine, American Academy of Physical Medicine and Rehabilitation, and Association of Academic Physiatrists Disclosure: Nothing to disclose. Daniel D Scott, MD, MA Associate Professor, Department of Physical Medicine and Rehabilitation, University of Colorado School of Medicine; Attending Physician, Department of Physical Medicine and Rehabilitation, Denver Veterans Affairs Medical Center, Eastern Colorado Health Care System Daniel D Scott, MD, MA is a member of the following medical societies: Alpha Omega Alpha, American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, American Paraplegia Society, Association of Academic Physiatrists, National Multiple Sclerosis Society, and Physiatric Association of Spine, Sports and Occupational Rehabilitation Disclosure: Nothing to disclose. Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference Disclosure: Medscape Salary Employment
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