Guillain-Barré syndrome

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Guillain-Barré Syndrome

 

Definition

Guillain-Barré syndrome (GBS) causes progressive muscle weakness and paralysis (the complete inability to use a particular muscle or muscle group), which develops over days or up to four weeks, and lasts several weeks or even months.

Description

The classic scenario in GBS involves a patient who has just recovered from a typical, seemingly uncomplicated viral infection. Symptoms of muscle weakness appear one to four weeks later. The most common preceding infections are cytomegalovirus, herpes, Epstein-Barr virus, and viral hepatitis. A gastrointestinal infection with the bacteria Campylobacter jejuni is also common and may cause a severe type of GBS from which it is particularly difficult to recover. About 5% of GBS patients have a surgical procedure as a preceding event. Patients with lymphoma, systemic lupus erythematosus, or AIDS have a higher than normal risk of GBS. Other GBS patients have recently received an immunization, while still others have no known preceding event. In 1976–77, there was a vastly increased number of GBS cases among people who had been recently vaccinated against the Swine flu. The reason for this phenomenon has never been identified, and no other flu vaccine has caused such an increase in GBS cases.

Causes and symptoms

The cause of the weakness and paralysis of GBS is the loss of myelin, which is the material that coats nerve cells (the loss of myelin is called demyelination). Myelin is an insulating substance which is wrapped around nerves in the body, serving to speed conduction of nerve impulses. Without myelin, nerve conduction slows or stops. GBS has a short, severe course. It causes inflammation and destruction of the myelin sheath, and it disturbs multiple nerves. Therefore, it is considered an acute inflammatory demyelinating polyneuropathy.
The reason for the destruction of myelin in GBS is unknown, although it is thought that the underlying problem is autoimmune in nature. An autoimmune disorder is one in which the body's immune system, trained to fight against such foreign invaders as viruses and bacteria, somehow becomes improperly programmed. The immune system becomes confused, and is not able to distinguish between foreign invaders and the body itself. Elements of the immune system are unleashed against areas of the body, resulting in damage and destruction. For some reason, in the case of GBS, the myelin sheath appears to become a target for the body's own immune system.
The first symptoms of GBS consist of muscle weakness (legs first, then arms, then face), accompanied by prickly, tingling sensations (paresthesias). Symptoms affect both sides of the body simultaneously, a characteristic that helps distinguish GBS from other causes of weakness and paresthesias. Normal reflexes are first diminished, then lost. The weakness eventually affects all the voluntary muscles, resulting in paralysis. When those muscles necessary for breathing become paralyzed, the patient must be placed on a mechanical ventilator which takes over the function of breathing. This occurs about 30% of the time. Very severely ill GBS patients may have complications stemming from other nervous system abnormalities which can result in problems with fluid balance in the body, severely fluctuating blood pressure, and heart rhythm irregularities.

Diagnosis

Diagnosis of GBS is made by looking for a particular cluster of symptoms (progressively worse muscle weakness and then paralysis), and by examining the fluid that bathes the brain and spinal canal through cerebrospinal fluid (CSF) analysis. This fluid is obtained by inserting a needle into the lower back (lumbar region). When examined in a laboratory, the CSF of a GBS patient will reveal a greater-than-normal quantity of protein, with normal numbers of white blood cells and a normal amount of sugar. Electrodiagnostic studies may show slowing or block of conduction in nerve endings in parts of the body other than the brain. Minor abnormalities will be present in 90% of patients.

Treatment

There is no direct treatment for GBS. Instead, treatments are used that support the patient with the disabilities caused by the disease. The progress of paralysis must be carefully monitored, in order to provide mechanical assistance for breathing if it becomes necessary. Careful attention must also be paid to the amount of fluid the patient is taking in by drinking and eliminating by urinating. Blood pressure, heart rate, and heart rhythm also must be monitored.
A procedure called plasmapheresis, performed early in the course of GBS, has been shown to shorten the course and severity of GBS. Plasmapheresis consists of withdrawing the patient's blood, passing it through an instrument that separates the different types of blood cells, and returning all the cellular components (red and white blood cells and platelets) along with either donor plasma or a manufactured replacement solution. This is thought to rid the blood of the substances that are attacking the patient's myelin.
It has also been shown that the use of high doses of immunoglobulin given intravenously (by drip through a needle in a vein) may be just as helpful as plasma-pheresis. Immunoglobulin is a substance naturally manufactured by the body's immune system in response to various threats. It is interesting to note that corticosteroid medications (such as prednisone), often the mainstay of anti-autoimmune disease treatment, are not only unhelpful, but may in fact be harmful to patients with GBS.

Prognosis

About 85% of GBS patients make reasonably good recoveries. However, 30% of adult patients, and a greater percentage of children, never fully regain their previous level of muscle strength. Some of these patients suffer from residual weakness, others from permanent paralysis. About 10% of GBS patients begin to improve, then suffer a relapse. These patients suffer chronic GBS symptoms. About 5% of all GBS patients die, most from cardiac rhythm disturbances.
Patients with certain characteristics tend to have a worse outcome. These include people of older age, those who required breathing support with a mechanical ventilator, and those who had their worst symptoms within the first seven days.

Prevention

Because so little is known about what causes GBS to develop, there are no known methods of prevention.

Resources

Organizations

American Academy of Neurology. 1080 Montreal Ave., St. Paul, MN 55116. (612) 695-1940. http://www.aan.com.
Guillain-Barré Syndrome Foundation International. PO Box 262, Wynnewood, PA 19096. (610) 667-0131. (610) 667-0131. http://www.webmast.com/gbs.

Key terms

Autoimmune — The body's immune system directed against the body itself.
Demyelination — Disruption or destruction of the myelin sheath, leaving a bare nerve. Results in a slowing or stopping of impulses traveling along that nerve.
Inflammatory — Having to do with inflammation, the body's response to either invading foreign substances (such as viruses or bacteria) or to direct injury of body tissue.
Myelin — The substance that is wrapped around nerves, and which is responsible for speed and efficiency of impulses traveling through those nerves.

Guillain-Barré syndrome

 [ge-yă´ bah-ra´]
a relatively rare disease affecting the peripheral nervous system, especially the spinal nerves, as well as the cranial nerves. Pathologic changes include demyelination, inflammation, edema, and nerve root compression. The cause is unknown; however, it usually follows a febrile illness such as respiratory infection or gastroenteritis within 10 to 21 days and is believed by some to be related to an autoimmune mechanism. Because it is characterized by a flaccid paralysis, it is sometimes mistaken for poliomyelitis. Called also acute idiopathic polyneuritis, acute postinfectious polyneuritis, and Landry's paralysis.

The early symptoms are fever, malaise, nausea, or prostration. Muscular weakness usually starts in the lower extemities and tends to go upward through the body, but it may affect the facial muscles and arms first and then move downward. The paralysis is not accompanied by loss of sensation, but rather by abnormal sensations of tingling and numbness. The classic cerebrospinal fluid findings are of an elevated protein level without an increase in the number of leukocytes. The cerebrospinal fluid pressure is within normal limits.

The progression of the paralysis may stop at any point. Once the weakness reaches its maximum, the paralysis remains unchanged for days or weeks. Improvement begins spontaneously and continues for weeks, or rarely, months. The prognosis for full recovery is good.

Guillain-Barré syndrome affects primarily the ventral roots of the spinal cord, hence the motor disturbances. The sensory counterpart of this syndrome affects the dorsal roots and is usually called Guillain-Barré-Strohl syndrome. The symptoms of this disease include severe stabbing pains at first, followed by abnormally exaggerated response to painful stimuli.
Patient Care. There is no specific treatment for the disease. It must run its course and for this reason skilled patient care is imperative, particularly in the acute phase, when respiratory failure requiring prolonged mechanical ventilation is a very real possibility. All measures needed to prevent complications in patients who cannot move about in bed are required. The experience of paralysis and sensory disturbances is an ordeal for them. They will need continued physical and psychological support throughout all stages of recovery, which may last for weeks or months, depending on the individual patient.

Guil·lain-Bar·ré syn·drome

(gē-yan[h]' bă-rā'),
an acute, immune-mediated disorder of peripheral nerves, spinal roots, and cranial nerves, commonly presenting as a rapidly progressive, areflexive, relatively symmetric ascending weakness of the limb, truncal, respiratory, pharyngeal, and facial musculature, with variable sensory and autonomic dysfunction; typically reaches its nadir within 2-3 weeks, followed initially by a plateau period of similar duration, and then subsequently by gradual but complete recovery in most cases. Guillain-Barré syndrome is often preceded by a respiratory or gastrointestinal infection and is associated with albuminocytologic dissociation of the cerebral spinal fluid. Although classically considered pathologically to be an acute, inflammatory demyelinating polyradiculoneuropathy (q.v.), pure axon degeneration forms recently have been recognized.

Guillain-Barré Syndrome

(gē-yăn′bə-rā′)
n.
A temporary inflammation of the nerves, causing pain, weakness, and paralysis in the extremities and often progressing to the chest and face. It typically occurs after recovery from a viral infection or, in rare cases, following immunization for influenza.

Guillain-Barré syndrome

[gēyan′bärā′]
Etymology: Georges Guillain, French neurologist, 1876-1951; Jean A. Barré, French neurologist, 1880-1967
an idiopathic, peripheral polyneuritis that may occur 1 to 3 weeks after a mild episode of fever associated with a viral infection or with immunization but that can also occur with no preceding illness. Symmetric pain and weakness affect the extremities, and paralysis may develop. The neuritis may spread to the trunk and face. Symptoms vary in intensity from mild to severe enough to require critical nursing care, including ventilator assistance. Treatment consists of supportive care and high IV doses of immunoglobulins. Recovery depends on the extent of neuritis and may take weeks to many months. Also called acute febrile polyneuritis, acute idiopathic polyneuritis, infectious polyneuritis.
observations Manifestations may range from mild to severe and generally develop 1 to 3 weeks after an upper respiratory or gastrointestinal infection. The first sign is symmetric muscle weakness in the distal extremities accompanied by paresthesia. This weakness spreads upward to the arms and trunk and then to the face. This ascension usually peaks about 2 weeks after onset. Deep tendon reflexes are commonly absent. Difficulty chewing, swallowing, and speaking may occur, and respiratory paralysis may develop. Signs of autonomic nervous system dysfunction, such as facial flushing, profuse diaphoresis, bowel and bladder atony, postural hypotension, hypertension, tachycardia, and heart block, may develop. Deep, aching muscle pain is also common. The diagnosis is based on history and clinical presentation. Lumbar puncture results typically reveal an increase in cerebrospinal fluid protein without an increase in lymphocyte count. Electromyography is markedly abnormal with reduced nerve conduction velocity. About 5% of those affected die of respiratory failure. Another 10% have permanent residual neurological deficits. About 90% of all survivors make a full recovery, but the recovery time may be as long as 3 years.
interventions Treatment is supportive, with the use of IV immunoglobulins or plasmapheresis to counteract neurological defect and speed recovery of neurological deficit. Subcutaneous heparin is given to prevent thromboembolism. Tracheostomy and mechanical ventilation are necessary to treat respiratory paralysis, and breathing function tests should be performed and followed closely. Continuous cardiac monitoring is done to detect possible sinus tachycardia and/or bradyarrhythmias.
nursing considerations Care for patients with Guillain-Barré disease is complex and multifaceted. In acute disease, nursing focus is on careful assessment of ascending paralysis and monitoring of respiratory function to ensure airway patency and adequate gas exchange. Continuing assessments are needed of corneal, gag, and swallow reflexes. Blood pressure is monitored for fluctuations; cardiac rate and rhythm are monitored for tachycardia, bradycardia, heart block, and asystole. Pain assessment and management are required for paresthesias, hyperesthesias, muscle cramps, and deep muscle aches. Complications related to autonomic dysfunction, paralysis, and immobility (e.g., pressure sores, thromboemboli, aspiration, urinary retention, fecal impaction, and nerve palsies) must be prevented. This includes a rigorous turning and positioning schedule, regular passive range-of-motion exercises, careful pulmonary toilet and feeding routines, application of thromboembolic stockings, and institution of bowel and bladder programs. Communication systems may be needed if the individual is on a ventilator or has facial paralysis. Emotional and social support are needed to reduce fear and anxiety. Rehabilitation may be indicated for recovery of functional abilities and long-term adaptation to permanent neurological deficit.

Guil·lain-Bar·ré syn·drome

(gē-ahn[h]' bah-rā' sin'drōm)
A self-limiting demyelinating syndrome related to autoimmune dysfunction, surgical complication, some vaccines, Hodgkin disease, and some types of drug reactions. Motor and/or sensory dysfunction begins in the extremities and moves proximally, sometimes leading to respiratory failure, before function is restored within weeks or months.
Synonym(s): Landry paralysis, Landry syndrome, polyradiculoneuritis.

Guillain-Barré syndrome

A serious immunological disorder affecting nerves in which damage occurs to the insulating fatty sheaths (myelin). The condition almost always follows a virus or bacterial infection. Cytomegalovirus, Epstein-Barr virus, Campylobacter jejuni and Mycoplasma pneumoniae have been implicated. Spinal roots are infiltrated with T cells and macrophages and the latter invade and strip off the myelin sheaths. In mild cases axons remain unaffected and remyelinization occurs; in severe cases axon degeneration also occurs. There is backache, tingling and numbness extending from the hands and feet to other parts of the body and progressive muscle weakness, sometimes amounting to complete paralysis. So long as the breathing is maintained, by artificial means if necessary, even these serious cases usually recover completely within two months. (Georges Guillain, French neurologist, 1876–1961 and Jean Alexandre Barré, French neurologist, 1880–1967).

Barré,

Jean A., French neurologist, 1880-1971.
Barré sign - a hemiplegic placed in the prone position with the limbs flexed at the knees is unable to maintain the flexed position on the side of the lesion but extends the leg.
Barré-Liéou syndrome - irritation of a nerve plexus around a vertebral artery produces symptoms of dizziness, headache, tinnitus. Synonym(s): Liéou-Barré syndrome
Guillain-Barré reflex - see under Guillain
Guillain-Barré syndrome - see under Guillain
Landry-Guillain-Barré syndrome - Synonym(s): Landry syndrome
Liéou-Barré syndrome - Synonym(s): Barré-Liéou syndrome

Guillain,

Georges, French neurologist, 1876-1961.
Guillain-Barré reflex - plantar flexion of the foot and toes elicited by tapping the sole near its outer edge. Synonym(s): aponeurotic reflex
Guillain-Barré syndrome - a syndrome marked by paresthesia of the limbs, muscular weakness or a flaccid paralysis, and increased protein in the cerebrospinal fluid without increase in cell count. Synonym(s): acute idiopathic polyneuritis
Landry-Guillain-Barré syndrome - Synonym(s): Landry syndrome

Guil·lain-Bar·ré syn·drome

(gē-ahn[h]' bah-rā' sin'drōm)
An acute, immune-mediated disorder of peripheral nerves, spinal roots, and cranial nerves, commonly presenting as a rapidly progressive, areflexive, relatively symmetric ascending weakness of the limb, truncal, respiratory, pharyngeal, and facial musculature, with variable sensory and autonomic dysfunction.

Guillain-Barré syndrome

a relatively rare disease of humans affecting the peripheral nervous system, especially the spinal nerves, but also the cranial nerves. Often observed after parenteral administration of drugs and vaccines. Pathological changes include demyelination, inflammation, edema and nerve root decompression. Called also acute idiopathic polyneuritis, postinfectious polyneuritis and Landry's paralysis. The cause is unknown but is believed to be related to an autoimmune mechanism. Idiopathic polyradiculoneuritis (coonhound paralysis) in dogs and cauda equina neuritis in horses are similar.