Place: Sara Burke
During my senior year of high school, around mid-December, I noticed my very good friend and significant other, Matt, walking in a peculiar manner. His movements were purposeful, but abrupt and forceful instead of smooth and continuous. Matt had a very difficult time climbing the stairs, often using the railing to try and pull himself along. His speech became slightly slurred, and he became weak, needing someone to carry his book bag for him constantly while he struggled to walk. Matt complained frequently about a painful tingly sensation in his limbs and numbness in his right hand and foot. Two weeks previous to the onset of these symptoms, Matt had been diagnosed and treated for a sinus infection by his doctor.
Matt’s unusual symptoms continued for one week, gradually worsening. Finally on a Friday afternoon, his mom picked him up from school and they returned to the doctor’s office. In the same night they visited the emergency room, and about four hours later Matt was diagnosed with Guillain-Barré Syndrome.
Guillain-Barré Syndrome (GBS) can affect any person, regardless of age, gender or race. “It is the most common cause of rapidly acquired paralysis in the United States today, affecting one to two people in every 100,000” (“Learning About…”). GBS is nonepidemic and nonseasonal, occurring more frequently in those between the ages of 30 and 50 years in the adult population (Monahan and Neighbors 773). Usually GBS occurs a few days or weeks after having had symptoms of a gastrointestinal, viral, or respiratory infection. Sporadically, vaccinations or surgery may also activate the syndrome (“Guillain-Barré…”). “…Guillain-Barré syndrome…can arise in approximately one in 100,000 [influenza virus] vaccine recipients” (Talaro and Talaro 773).
Guillain-Barré Syndrome is an autoimmune disease in which the body’s immune system attacks nerves outside of the spinal cord and brain, also known as the peripheral nerves. Under normal body conditions, the cells of the immune system attack only materials and organisms detected by the body as foreign. However, with Guillain-Barré, the immune system attacks part of the peripheral nerves as if they were foreign to the body. In particular, the myelin sheath is destroyed. Myelin sheath is an insulating layer of lipid fat and protein that covers many peripheral nerve axons, allowing for rapid transmission of nerve impulses (“Learning About…”).
Guillain-Barré Syndrome is characterized by a rapid onset of weakness, tingling, or very painful burning sensations. Sensory reception and simple reflexes are lost initially, an early cue towards diagnosis (“Learning About…”). The erosion of the myelin sheath commonly begins from the distal end of the legs and arms and moves towards the upper body. Because the myelin sheath increases the rate of nervous signal transmission, its degradation causes inefficient conduction. Because many peripheral nerves are long, they are especially vulnerable to interruption. Muscles begin losing their ability to respond to the brain’s commands. At the same time, the brain is receiving fewer sensory signals from the affected areas, resulting in the inability to feel pain, heat, and other sensations. These symptoms increase in intensity until the muscles become useless and the patient feels almost paralyzed (“Guillain-Barré…”). “The individual may have a symmetric weakness or paralysis involving the legs, the trunk, and possibly the neck and face” (Huether and McCance 565). Once the demyelination reaches the upper body, specifically the heart and lungs, breathing rhythmicity and heart rate are vulnerable to impairment. At this point, the patient’s state is a severe medical emergency. These symptoms can progress over different periods of time depending on the person, but most patients reach the greatest stage of weakness within two weeks, and by the third week 90% of all patients are at their weakest (“Guillain-Barré…”).
The cause of Guillain-Barré Syndrome is unknown, although it is widely accepted as an autoimmune response. Campylobacter jejuni, a common cause of diarrheal illness in developed countries, is believed to cause an immune response against the own body’s peripheral nerves (Monahan and Neighbors 773). “When Guillain-Barré is preceded by a viral or bacterial infection, it is possible that the virus has changed the nature of cells in the nervous system so that the immune system treats them as foreign cells. It is also possible that the virus makes the immune system itself less discriminating about what cells it recognizes as its own, allowing some of the immune cells, such as certain kinds of lymphocytes [T&B] and macrophages , to attack the myelin” (“Guillain-Barré…”).
The diagnosis of Guillain-Barré Syndrome can be a tricky task, since many disorders have similar symptoms. Primarily, diagnosis is based on physical examination and history. A lumbar puncture (spinal tap) can be performed for cerebrospinal fluid analysis. An escalated protein count without noteworthy cell count increase is common of Guillain-Barré patients. Also, nerve conduction studies can be performed, showing abnormalities in nerve transmission after the onset of symptoms (Monahan and Neighbors 773).
There is no known cure for GBS, but there are therapies that lessen the severity of the illness and accelerate recovery in most patients. “Treatment focuses on supportive therapy and prevention of complications, such as pneumonia and respiratory failure secondary to respiratory paralysis and cardiovascular complications resulting from paralysis and immobility” (Monahan and Neighbors 773). Often caregivers may be instructed to manually move the limbs of the patient to help improve muscle stimulation during recovery, and help keep the muscles strong and flexible (“Guillain-Barré…”). An old saying could not be truer here…if you don’t use it, you lose it.
One common therapy for GBS patients is plasma exchange (plasmapheresis). During plasmapheresis, whole blood is removed from the body and processed so that both white and red blood cells separate from the plasma. The blood cells are returned without the plasma, which the body rapidly regenerates. Although many scientists are unsure of the benefits of plasmapheresis, it seems to shorten the duration of the GBS period. It is thought that the plasma portion of the blood may contain elements toxic to the myelin sheath, and thus when removed slow down the demyelination process (“Guillain-Barré…”).
Another therapy which is more easily conducted than plasma exchange is high-dose immunoglobin therapy. These immune system proteins are given by intravenous injection to reinforce the immune system and attack foreign organisms. This also reduces the severity of symptoms, but the reason why this therapy works is also unknown (“Learning About…”).
The patient is commonly treated in an intensive or critical care unit in a hospital where plasma exchange or immunoglobin therapy is quickly accessible. As his/her condition improves, the patient may be moved to a different care unit to begin their recovery. Recovery is not necessarily immediate, lasting from as little as a few weeks to a few years, depending on the level of damage when treatment begins. In the peripheral nervous system, if the nerve cell body survives, regeneration takes place and motor function is likely to be recovered. If the nerve cell body dies from intense ventral root association with the degenerative process, no regeneration is possible (Huether and McCance 565). “Remyelination and regeneration of axons occurs spontaneously and completely in approximately 80% of cases” (Monahan and Neighbors 773). “About 30% of those with Guillain-Barré still have a residual weakness after 3 years. About 3% may suffer a relapse of muscle weakness and tingling sensations many years after the initial attack” (“Guillain-Barré…”).
Some patients require psychological counseling as part of their treatment. This is due to the great emotional and physically stress caused by the quick onset of symptoms and the level of seriousness associated with the syndrome. Paralysis certainly does not float lightly on the minds of many people, and to be suddenly unable to move can be very shocking. To know that one’s bodily condition is worsening so quickly with potentially severe consequences can be emotionally devastating. Some patients require assisted care pre and/or post treatment, and this can be emotionally and psychologically burdening as well (“Guillain-Barré…”). Physical therapy, commonly strength exercises, should be pursued when residual motor problems are present (Monahan and Neighbors 773).
Currently, scientists are concentrating their efforts on developing new treatments and refining existing ones. They are also studying the immune system to identify the cells responsible for beginning and advocate the nervous system rampage. Because so many cases of Guillain-Barré Syndrome begin after a viral or bacterial infection, speculations are that certain characteristics of some viruses and bacteria may improperly activate the immune system. Certain proteins or peptides common to myelin may be the same as those in viruses or bacteria, causing an attack on the myelin itself (“Guillain-Barré…”).
Matt was found to be contagious with strep throat when admitted to the hospital. It was a strain that went undetected at the doctor’s office but was detectable at the hospital. The strep infection, which had gone untreated for an unknown period before his arrival, was believed to be the stimulus behind the onset of his Guillain-Barré flare-up.
Matt was treated in intensive care for five days. During this time he endured a spinal tap analysis, two nerve conduction studies and many blood drawing sessions. Matt’s treatment was plasma exchange therapy with which he was treated for five days. After the treatment he was moved to progressive care where he spent three days learning how to walk and speak again. He retrained his lower limb muscles to endure the stresses of weight support and balance. After his release from the hospital he continued his prescribed recovery through four months of physical and speech therapy.
Today, four years later, Matt still has difficulty pronouncing particular words correctly, but has recovered, he says, about 90%. He continues his physical therapy at home regularly, and is grateful to have made a wonderful recovery.
Syndrome Fact Sheet.” Office of Communications and Public Liaison,
National Institute of Neurological Disorders and Stroke, National Institutes of Health.
Sue E., and Kathryn L. McCance. Pathophysiology: The Biologic Basis for
Disease in Adults and Children. Third Edition.
Talaro, Arthur, and Kathleen Park Talaro. Foundations in Microbiology. Third Edition. WCB / McGraw-Hill, 1999.