| Guillain-Barré syndrome (GBS) is an inflammatory disease of the peripheral nervous system, those nerves outside the brain and spinal cord. As the nerves become inflamed, both movement (motor), and sensation (sensory) functions are impaired. This syndrome is characterized by the rapid onset of weakness, sensory loss, and impairment of reflexes; often paralysis of the legs, arms, breathing muscles, and face develops. Because it involves more than one nerve, it is considered a polyneuropathy.
In two-thirds of individuals, a mild respiratory or gastrointestinal infection precedes the symptoms by 1 to 3 weeks or longer (Fanion). Studies show that a certain type of bacteria called Campylobacter jejuni is the most frequent preceding infection. Other less frequent preceding events or illnesses include surgical procedures, other bacterial infections, exposure to thrombolytic agents, or lymphoma (Hodgkin's disease). Some case reports have associated heavy metal poisoning as an etiology. Some theories suggest an autoimmune mechanism in which the individual's defense system of antibodies and white blood cells are triggered into damaging the nerve covering (myelin), leading to weakness and abnormal sensation. Vaccinations have also been associated with GBS. The administration of rabies vaccine and the 1976 swine influenza vaccine was associated with an increase in the incidence of Guillain-Barré syndrome.Risk: Guillain-Barré syndrome occurs in all parts of the world and in all seasons. It affects adults of all ages and both sexes, although males seem to be slightly more susceptible to the disease. Attack rates are highest in individuals 50 to 74 years of age. The death rate is 1.3 times higher in males than females with symptom onset after 40 years of age (Cha-Kim). Incidence and Prevalence: The annual incidence in the US is similar to the incidence worldwide, varying from 0.6 to 3 per 100,000 individuals (Sater; Cha-Kim). |
Source: Medical Disability Advisor
| History: Guillain-Barré syndrome is easily recognized. Tingling and burning sensations (paresthesias) and numbness are frequently reported by individuals, although these symptoms may be absent in a few cases. Most individuals report progressive weakness, starting in the limb muscles farthest from the point of origin (distal); it generally develops in the legs before the arms or the trunk, and spreads to the neck and cranial muscles later. The weakness can progress to total motor paralysis, and occurs over days to weeks with peak deficits reached by 4 weeks (Cha-Kim). More than 50% of individuals report pain and an aching discomfort in the muscles that are more severe at night, especially in the shoulder girdle, hips, posterior thighs, and back (Newswanger). Occasionally facial, eye (ocular), or throat (oropharyngeal) muscles are affected. Individuals may report difficulty swallowing (dysphagia), visual disturbances, or speech impairment (dysarthria). Physical exam: Initially reflexes are reduced, and eventually become absent. In rare cases, only the ankle reflexes are lost during the first week of the illness. In the early stage, arm muscles will be less weak than the leg muscles, or they may be spared entirely. Disturbances of the autonomic (functionally independent) nervous system including facial flushing, rapid heartbeat (tachycardia), alternating high blood pressure (hypertension) and low blood pressure (hypotension), and either loss of sweating or profuse sweating (diaphoresis) are commonly found but usually do not persist longer than a week or 2. Cranial nerve involvement is present in 45% to75% of individuals (Cha-Kim). Urinary retention occurs in about 15% of individuals. Tests: Electrodiagnostic tests, including electromyography (EMG) and nerve conduction velocity (NCV), that demonstrate significantly slowed or blocked impulses support the diagnosis. Slowed F-wave responses are the first abnormality chronologically found in NCV testing. Otherwise, NCV tests may be normal for 2 to 3 weeks. Nerve conduction velocity tests show sensory abnormalities in 58% to 76% of individuals with GBS (Cha-Kim). A lumbar puncture is performed and a sample of cerebral spinal fluid (CSF) is analyzed. Elevated protein in the CSF is seen in most individuals, but may be normal the first few days after onset. The CSF cell count is usually normal, but in about 10% of cases there may be an elevated white blood cell count. Precursor viral infections may also be documented by blood tests. |
Source: Medical Disability Advisor
| Because progression of this disease in its early stages is unpredictable, most individuals are initially hospitalized in an intensive care unit (ICU) so breathing and other body functions can be monitored. The treatment for acute, severe cases of Guillain-Barré is respiratory assistance and careful nursing. Approximately one-third of individuals may require a respirator (ventilation) for breathing (Cha-Kim). Individuals with swallowing weakness (dysphagia) may require intubation to prevent aspiration of food particles. Other major components of therapy include support of blood pressure by volume infusion, administration of medication to manage low blood pressure, and prevention of sodium, potassium, and chloride (electrolyte) imbalance. Only extreme cases of high blood pressure (hypertension) require treatment with anti-hypertensive agents.
Blood plasma exchange (plasmapheresis) has been found to be effective in severe cases. In this procedure, blood is withdrawn and the plasma is removed and replaced with fresh frozen plasma or albumin. Intravenous (IV) administration of immunoglobulin is as effective as plasma exchange; it is also safer and more immediately available. Individuals who are treated within 2 to 4 weeks of onset experience a distinct reduction in hospitalization time and better recovery of walking ability. There is no additional benefit from administering both IV immunoglobulin infusion and plasmapheresis together.
Physical therapy should be started as soon as the individual is able to tolerate it to reduce recovery time. |
Source: Medical Disability Advisor
| About 85% of individuals completely recover from the condition, and about 7% to 15% recover with severe residual effects; between 2% and 5% do not survive the illness (Newswanger; Cha-Kim). In the early stages, death is often due to cardiac arrest, but later in the illness, pulmonary embolism and other complications, such as infection, are the main causes. Of those individuals who recover with severe residual effects, most had either a severe form of the disease, widespread nerve damage, or required prolonged mechanical ventilation. Individuals who recover completely either have no residual effects or mild motor deficits in the legs or feet.
Recovery usually occurs within a few weeks or months, unless there is severe nerve damage; then it takes 6 to 18 months to regenerate the nerves. Up to 20% of individuals are unable to walk unaided by 6 months (van Doorn). Recovery is maximal by 18 months (Newswanger). Older individuals recover more slowly than younger ones and are more likely to have residual weakness. Individuals who have pronounced sensory loss and imbalance are likely to have these difficulties indefinitely. About 5% to 9% of individuals will experience one or more recurrences of polyneuropathy. In these cases, the nerves may become enlarged and the neuropathy may become chronic; this should be distinguished from acute Guillain-Barré syndrome. |
Source: Medical Disability Advisor
| Individuals with Guillain-Barré syndrome may require respiratory, speech, physical, and occupational therapy, depending upon the extent of disability. Individuals may also require psychological counseling to help cope with the recent onset of disability and to maintain motivation for rehabilitation.
Inpatient respiratory therapy may be required if the individual presents with weakness of the diaphragm and the muscles of the ribcage (intercostal muscles). Respiratory therapists teach individuals to use an incentive spirometer to help support respiration and decrease the risk of pulmonary complications, and instruct in how to produce an effective cough to prevent the build up of secretions in the lungs. For individuals who have severely weakened respiratory muscles, a ventilator may be required temporarily.
Individuals require inpatient speech therapy if they are on a ventilator. Speech therapists can supply the individual with a communication board so that they are able to maintain contact with their family and health care professionals. When the ventilator is no longer needed, speech therapists help individuals learn how to strengthen swallowing muscles.
Individuals require physical and occupational therapy on an inpatient as well as an outpatient basis. Rehabilitation needs vary among individuals, as do rehabilitation treatments. Initially, therapy goals are more supportive than rehabilitative. Occupational and physical therapists focus on preventing joint contractures through passive and active range of motion exercises to all joints. All active range of motion exercises should be performed without compromising endurance to prevent further damage to the nervous system. Additionally, therapists work with individuals, their families, and the nurses to initiate a turning and positioning schedule for the prevention of pressure sores (decubitus ulcers).
Once individuals are stabilized and function has begun to return, inpatient rehabilitation can begin. Individuals begin a submaximal strengthening program in occupational and physical therapy for the arms, legs, and trunk to maintain functional abilities, and progress to functional strengthening to support transfer ability and later gait retraining. All strengthening exercises are performed to the individual's tolerance to prevent fatigue.
Individuals may have impaired coordination. Occupational therapists instruct individuals to perform fine motor coordination exercises, self-care activities, and community re-integration tasks. Physical therapists instruct individuals in gross motor coordination and proprioceptive tasks. In both physical and occupational therapy, individuals work on static and dynamic sitting and standing balance. Physical therapists also begin gait retraining once the individual becomes stronger, first using the parallel bars and then progressing to independent ambulation as able.
The main focus of physical and occupational therapy is to maximize functional capabilities. Occupational therapists teach individuals skills such as getting in and out of the shower, dressing, and preparing meals. Occupational therapists may order adaptive equipment or braces to make these tasks easier. Physical therapists teach skills such bed mobility, transfers, walking, or using a wheelchair if needed. Physical therapists may order assistive devices such as crutches or a cane; self-propelled or power wheelchairs if necessary; and may also refer an individual to an orthotist if leg braces are deemed appropriate. |
Source: Medical Disability Advisor
| Complications include blood clots to the lung (pulmonary embolism), partial or complete collapse of a lung (atelectasis), tracheal erosion, respiratory infection such as aspiration pneumonia, increased intracranial pressure, additional infections such as infections of the urinary tract (UTI) or skin (cellulitis), and psychological stress. |
Source: Medical Disability Advisor
| Extended leave from work may be required for individuals with respiratory complications, severe residual effects from the condition, and for those who need physical therapy during recovery. Even for those who recover with few residual side effects, work duties requiring minimal physical activity and mobility may be necessary for a while. |
Source: Medical Disability Advisor
| If an individual fails to recover within the expected maximum duration period, the reader may wish to consider the following questions to better understand the specifics of an individual's medical case. Regarding diagnosis:
- Were other neuropathies and spinal cord diseases (other types of acute polyneuropathy, acute spinal cord disease, acute myasthenia gravis, tick paralysis, hexacarbon abuse, acute alcoholic neuropathy, basilar artery thrombosis or embolism, neuromuscular junction disease, "locked-in" syndrome, chronic demyelinating polyradiculopathy with acute presentation, acute pandysautonomia, acute transverse myelopathy, botulism poisoning, poliomyelitis, saxitoxin poisoning, diphtheria, and heavy metal intoxication) ruled out?
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Has diagnosis of Guillain-Barré syndrome been confirmed? Based on what criteria?
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Has individual experienced any complications, such as pulmonary embolism, atelectasis, tracheal erosion, respiratory infection such as aspiration pneumonia, increased intracranial pressure, or additional infections such as infections of the urinary tract or cellulitis, and psychological stress?
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Does individual have an underlying condition, such as peripheral neuropathies, that may impact recovery?
Regarding treatment:
- Did individual require mechanical ventilation or intubation?
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Has independent function been restored?
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Were therapies such as plasma exchange or IV immunoglobulin infusion utilized? With what result?
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Was physical therapy initiated at the appropriate time?
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Would individual benefit from additional rehabilitation?
Regarding prognosis:
- Does individual have residual impairment? To what degree is function impacted?
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How old is individual?
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How long has Guillain-Barré syndrome been present?
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Have more than 18 months passed?
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Has sensory loss and imbalance resolved to any degree during that time?
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Has individual experienced recurrence of polyneuropathy?
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Has individual experienced a relapse?
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Has condition responded to treatment?
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Source: Medical Disability Advisor
| Cha-Kim, Angela. "Guillain-Barré Syndrome." eMedicine. Eds. Daniel D. Scott, et al. 14 Aug. 2004. Medscape. 30 Oct. 2004 <http://emedicine.com/pmr/topic48.htm>.Fanion, David, and Daniel M. Joyce. "Guillain-Barré Syndrome." eMedicine. Eds. Edward A. Michelson, et al. 14 Aug. 2004. Medscape. 30 Oct. 2004 <http://emedicine.com/emerg/topic222.htm>. Newswanger, D. L., and C. R. Warren. "Gullain-Barré Syndrome." American Family Physician 69 10 (2004): 2405-2410. MD Consult. 15 May. 2004. Elsevier, Inc. 30 Oct. 2004 <http://home.mdconsult.com/das/journal/view/42051340-2/N/14720246?sid=289778082&source=MI>. Sater, Richard A. "Acute Inflammatory Demyelinating Polyradiculoneuropathy." eMedicine. Eds. Donald B. Sanders, et al. 11 Nov. 2004. Medscape. 30 Oct. 2004 <http://emedicine.com/neuro/topic7.htm>. van Doorn, P. A., and R. van Koningsveld. "Immunotherapy for Guillain-Barré Syndrome." Lancet Neurology 3 2 (2004): 84-84. MD Consult. 1 Feb. 2004. Elsevier, Inc. 30 Oct. 2004 <http://home.mdconsult.com/das/journal/view/42051340-2/N/14336464?sid=289778082&source=MI>. |
Source: Medical Disability Advisor
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