John Dewey presents to the ED with the chief complaint of sudden loss of physical function. He's unable to move his legs and arms, and he has slight difficulty speaking. He tells you he had flu symptoms 3 weeks ago for which he took over-the-counter medications. Most of these symptoms disappeared 1 week ago, but he continued to feel progressively weaker.
Mr. Dewey's computed tomography scan shows no acute changes and his labwork is normal. Physical assessment reveals almost complete paralysis of the legs and arms, and loss of knee-jerk reflexes. Neurology was consulted and he was admitted to the ICU with a diagnosis of Guillain-Barré syndrome (GBS).
GBS is also called acute inflammatory demyelinating polyneuropathy and Landry ascending paralysis. According to the National Institute of Neurological Disorders and Stroke, it's defined as an inflammatory disorder of the peripheral nerves (those outside the brain and spinal cord). It's characterized by the rapid onset of weakness and, often, paralysis of the legs, arms, breathing muscles, and face.1,2
No one knows for sure what causes GBS. It's a rare disorder, with a frequency of about 1 to 2 cases per every 100,000 people per year in the United States. Healthy men and women, young and old, are equally prone to contracting it. Most commonly however, the ascending progression of loss of physical functions follows from a recent respiratory and/or gastrointestinal infection.1,2
The disorder is believed to be an autoimmune disease (a condition in which the immune system attacks its own tissues as though they were foreign substances). In response to a viral or bacterial infection or other illness, the immune system produces antibodies to fight the infection or illness; however, it may also produce antibodies that attack the covering (myelin sheath) of the peripheral nerves and sometimes the nerve fibers (axons), causing nerve damage. The resulting nerve damage leads to tingling and numbing sensations, muscle weakness, and paralysis.1,2
The American Academy of Neurology (AAN) has established some guidelines for diagnosing GBS.
Required features include:
- progressive weakness of more than one limb, ranging from minimal weakness of the legs to total paralysis of all four limbs, the trunk, bulbar (eyeball) and facial muscles, and external ophthalmoplegia (paralysis of the one or more extraocular muscles responsible for eye movements)
- distal areflexia with hyporeflexia at the knees and biceps if other features are consistent.
Supportive features include the following:
- progression of symptoms up to 4 weeks
- relative symmetry
- mild sensory symptoms or signs
- cranial nerve involvement, especially bilateral facial nerve weakness
- recovery starting 2 to 4 weeks after progression halts
- autonomic dysfunction
- no fever at the onset
- elevated protein in cerebrospinal fluid (CSF) with a cell count less than 10 mm3
- electrodiagnostic abnormalities consistent with GBS.
The following features make GBS diagnosis doubtful:
- sensory level (decrement or loss of sensation below a spinal cord root level as determined by neurologic exam)
- marked, persistent asymmetry of weakness
- severe and persistent bowel and bladder dysfunction.
- More than 50 white cells in the CSF.2
Let's go back to Mr. Dewey. After informed consent from his wife, Mr. Dewey received plasmapheresis. In this procedure, blood is removed from the body and processed so that the red and white blood cells are separated from the plasma, or liquid portion of the blood. The blood cells are then returned to the patient without the plasma, which the body quickly replaces.
Three to five exchanges of 50 mL/kg of plasma I.V. over 1 to 2 weeks via central venous catheter is suggested for GBS patients. It's also important to note that plasma exchange requires skilled personnel and specialized equipment that may not be available in all hospitals. In theory, plasma exchange may increase the risk of infection and hemorrhage as a result of the removal of immunoglobulins and clotting factors. Complications and adverse reactions from plasma exchange include hypotension, septicemia, pneumonia, cardiac arrhythmias, malaise, hypoprothrombinemia with bleeding/abnormal clotting, and hypocalcemia.3–6
Mr. Dewey also received intravenous immunoglobulin (IVIG) treatment, which uses fractionated, purified human plasma collected from a large pool of multiple donors. IVIG may work via several mechanisms, including the blockage of macrophage receptors, the inhibition of antibody production, the inhibition of complement binding, and the neutralization of pathologic antibodies. Manufacturer recommended dosing is 2 g/kg I.V., generally divided over 5 days. Some centers administer IVIG over 2 days. Adverse reactions to IVIG are usually minor and may include headache, fever, chills, malaise, and myalgia. Less common adverse reactions include migraines, aseptic meningitis, pulmonary edema, skin reactions (urticaria, pruritus, and petechia), and renal complications. Serum viscosity increases with IVIG therapy, which can result in thrombotic events such as stroke, pulmonary embolism, and myocardial infarction at 1 g/kg/day, especially in younger patients with normal renal and cardiovascular functions.3–6
Overall, about 70% of patients respond to either plasmapheresis or immunoglobulin. Although there's no evidence of additional benefit from treatment with both procedures, the AAN provides guideline recommendations.
According to the AAN:
- Plasma exchange is recommended for nonambulatory adult patients with GBS who start treatment within 4 weeks of the onset of neuropathic symptoms.
- Plasma exchange is also recommended for ambulatory patients who start treatment within 2 weeks of the onset of neuropathic symptoms.
- IVIG is recommended for nonambulatory adult patients with GBS who start treatment within 2 or possibly 4 weeks of the onset of neuropathic symptoms.
Nursing staff need to closely monitor the patient's breathing pattern to quickly identify the need for mechanical ventilation. Approximately one-third of patients require admission to an ICU, primarily because of respiratory failure. You'll need to feed the patient through a nasogastric tube due to loss of swallowing reflexes.
Routine performance of passive range of motion exercises will help keep the patient's muscles flexible. Involve physical therapists (PTs) and occupational therapists (OTs) as soon as the patient regains muscle control.7
It's also important to address your patient's psychological needs. Remember, he or she was most likely an active individual who suddenly lost control. Because of this shift, the patient might suffer depression.
Patients with GBS may remain in the hospital for several months, and recovery may take a year or more. It takes an interdisciplinary team to care for patients like Mr. Dewey. Physicians manage his care and ensure he remains medically stable. PTs offer progressive therapy goals to prevent muscle dystrophy. OTs focus on Mr. Dewey's upper mobility and activities of daily living.
Patients may express frustration when personal goals aren't met. Sometimes this frustration can be expressed with increased demand for staff attention. Family involvement is vital, as some patients are unable to resume their normal daily activities or occupation. Most patients with GBS recover completely, but some have residual weakness, numbness, and occasional pain.
Although Mr. Dewey hasn't returned to premorbid condition, he's able to move around using a motorized wheelchair and can feed himself. His family is supportive and ready to assist with home therapy.
2. Criteria for diagnosis of Guillain-Barré syndrome. Ann Neurol
3. Davids HR, Oleszek JL, Cha-Kim A. Guillain-Barré syndrome: treatment medication
4. Hadden RD, Cornblath DR, Hughes RA, et al. Electrophysiological classification of Guillain-Barré syndrome: clinical associations and outcome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Ann Neurol
5. Van der Meche FG, Schmitz PI. A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barre syndrome. Dutch Guillain-Barre Study Group. N Engl J Med
6. Griffin JW. Peripheral neuropathies: Immune-mediated neuropathies. In: Goldman L, Ausiello D, eds. Cecil Textbook of Medicine
. Vol 2. 22nd ed. Philadelphia, PA: Saunders; 2004:2379–2387.
7. Khan F. Rehabilitation in GBS. Aust Fam Physician