Alhtough none of the editors of Insidesurgery.com are participating in his care, we have noted the news reports that former Chicago Bears defensive lineman William “The Refrigerator” Perry has been hospitalized for Guillain Barre syndrome. He is listed as being in serious condition at Aiken Regional Medical Center in Aiken, South Carolina.
Family members have commented to the press that he is expected to recover.
News reports are incorrectly describing Guillain Barre syndrome (GBS) as a chronic inflammation of the peripheral nerves. Guillain Barre is an acute demyelinating disorder of the nervous system that is polyradiculopathic in nature (affects all nerves.) It was first described in 1916 by Guillain.
It is the most common cause of rapidly progressive weakness and occurs at .6 – 2.0 cases per 100,000 population. It affects all ages and the cause is unknown.
Guillain Barre syndrome usually presents 2 to 4 weeks after an infectious disease process such as the flu or a diarrheal illness. Causative agents of this prior illness are Epstein-Barr virus, cytomegalovirus (CMV), mycoplasma, chlamydia, herpes simplex, and Campylobacter jejuni. Guillain Barre was was a complication of the swine flu vaccine that was given to the United States public in 1976.
It has also been linked to an early presentation of HIV infection, immunization, general surgery, Hodgkin’s disease, kidney transplantation, and systemic lupus erythematosus.
Lyme disease can produce a syndrome of polyradicular weakness that may mimic GBS.
The hallmark of disease onset is the development of dysesthesias (painful “pins and needles”) in the hands and/or the feet.
The most striking feature of the syndrome is weakness that evolves over days to weeks and that in most cases ascends from the legs to the arms and head. In severe cases, the muscles of respiration can become paralyzed. There are rare variants of GBS where the paralysis begins in the upper regions of the body and descends.
About half of patients with GBS experience the full extent of their weakness in 14 days and 90% have reached their nadir by 4 weeks.
If the polyradiculopathy progresses longer than 8 weeks, the syndrome is differentiated through prognosis (outcome) from GBS and is termed chronic inflammatory demyelinating polyradiculopathy.
The severity of weakness in GBS is variable with some patients progressing to full paralysis of the affected body parts and some experiencing only minor weakness.
The weakness is usually symmetrical (identical on both sides) and there is loss or decrease in deep tendon reflexes. If the disease is severe in the later stages there may be fasciculations due to loss of axons.
In most cases, sensory loss is minor, although there is a variant form where sensory loss is profound. Respiratory failure is common and between 10% and 25% require ventilatory support (need to be placed on a breathing machine) due to paralysis of the muscles of breathing.
Weakness of the tongue muscles and muscles of deglutination (muscles of eating) is common. Involvement of the extraocular muscles (muscles that control eye movements) is uncommon, except in the Miller-Fischer variant.
Disturbances in the autonomic system are experienced in 50% of cases and can involve the sympathetic and/or parasympathetic nervous systems. Particularly worrisome findings include cardiac arrhythmias such as sinus tachycardia (fast heart rate), bradycardia (slow heart rate), ventricular tachycardia (can be lethal), atrial flutter, atrial fibrillation, and asystole (cardiac arrest).
Other autonomic disturbances in GBS include orthostatic hypotension (drop in blood pressure when the patient stands or the head is elevated), persistent hypertension (high blood pressure), bladder paralysis, abnormal sweating, and paralytic ileus (loss of bowel action.)
Laboratory and Test Diagnosis
The most characteristic laboratory finding of Guillain Barre syndrome is an abnormal elevation after 48 hours of symptom onset in the protein level of the cerebrospinal fluid (CSF) in the absence of significant pleocytosis (increase in cell count). However, not all patients show increased protein and the diagnosis must be made on other clinical features of the disease.
Nerve conduction studies are usually abnormal in GBS and show slowing of nerve conduction velocity with increases in distal motor and sensory latencies. Secondary to axonal loss or nerve conduction block, the amplitude of evoked motor responses is usually decreased.
It is not uncommon for routine nerve conduction studies on first testing to be relatively normal as the disease may be confined to the proximal nerve roots. However, upon closer scrutiny, the F-responses are usually found to be prolonged.
Laboratory analysis of the blood is surprisingly normal in GBS, with the exception of an usually mild increase in erythrocyte sedimentation rate (sed rate) and hyponatremia (low serum sodium). The disturbance in sodium levels is due to abnormal peripheral volume receptors leading to a syndrome of inappropriated anti-diuretic hormone secretion (SIADH), which raises blood volume and dilutes the sodium molecules present in the blood volume.
The pathological hallmark of Guillain Barre syndrome is demyelination of peripheral and cranial nerves. It is likely that both the humoral (B-cell) and cellular (T-cell) components of the immune system are involved in activating macrophages that cause the demyelination. The exact antigens that direct the immume response have not been identified.
It is now known that a variant of GBS is also marked by acute axonal loss of the motor and/or sensory nerves. Patients with sensory axonal loss have a more severe course and longer time to recovery.
The natural history of Guillain Barre syndrome is variable and depends on part on the severity of the symptoms. Generally, the disease is one of a natural, slow improvement from the state of maximum weakness, although deaths are not rare from the disease.
The average time to walk unassisted for most patients is 3 months from maximum weakness. The time for unassisted ambulation (walking) in patients who were ventilator dependent is 6 months.
The most powerful predictor of poor outcome is a mean distal motor amplitude of less than 20% of normal as seen on motor nerve conduction studies.
Other prognostic variables include age greater than 60 years, respiratory failure requiring ventilatory support, and rapidity of decline.
The mortality rate of GBS ranges from 3% to 8%. Approximately 15% of patients recover with no residual effects. Fifty to sixty percent of patients recover to near normal levels with mild defects. Loss of ambulation occurs in about 10% of patients.
It is difficult to predict which GBS patients will require ventilatory support based on the degree of weakness. The astute clinician will manage need for a ventilator expectantly by following serial forced vital capacity (a measure of how strongly a patient can blow out) and maximum inspiratory pressure.
Generally a FVC of 50% of normal will require oxygen and close observation. It is acceptable to intubate that patient based on clinical examination alone if the patient appears to be in respiratory distress.
The autonomic disturbances are evaluated through close monitoring. Hypotension (low blood pressure) is treated with fluid administration. Hypertension is treated with short-acting alpha blocking agents such as hydralazine. Tachycardia is treated with beta blockers or calcium channel blockers and bradycardia can be treated with atropine.
Hyponatremia is not uncommon and should be treated with administration of normal saline solution or sodium supplementation.
Almost all patients with Guillain Barre syndrome should be considered for plasmapheresis, which is a process of withdrawing the plasma component of blood and filtering it. The current recommendations are exchanging 200-250 ml of plasma per kg body weight in three to five treatments over one to two weeks. Plasmapheresis is most effective if given early in the course.
Until recently, the typical treatment regimen used banked fresh frozen plasma (FFP) as the exchanging fluid for the patient’s plasma. Because of the risks of transfusing FFP, the current recommendations of the Guillain Barre Study Group and the French Cooperative Group are for albumin to be used in lieu of banked plasma. Gamma globulin should be transfused after each exchange to prevent infection.
Human immunoglobulin (HIG) may also be considered for use in treatment of GBS and is thought to be roughly equivalent in efficacy (effect). It can be used in patients who can not tolerated the hypotension resulting from plasmapheresis or who do not have intravascular access.
HIG is associated with side effects in 3% – 12% of patients. These range from flu-like symptoms to aseptic meningitis, anaphylactic reactions in IgA deficient patients, and acute renal failure. Many clinicians prefer to begin treatment of GBS with human immunoglobulin due to its ease of administration. There may be a slight treatment advantage in combining HIG and plasmapheresis although the data are mixed on this strategy.
Counterintuitively, corticosteroids are not used in the treatment of Guillain Barre syndrome.