Guillain Barre' Syndrome

Guillain Barre' Syndrome
(Acute Idiopathic Polyneuritis)

Guillain-Barré (Ghee-yaw Bah-ray) Syndrome, also called acute inflammatory demyelinating polyneuropathy and Landry's ascending paralysis, is a disorder of the peripheral nerves, those outside the brain and spinal cord (peripheral nerves and spinal roots are the major sites of demyelination in GBS patients). It is typically characterized by the rapid onset of muscle weakness and often, paralysis of the legs, arms and breathing muscles. The cause of Guillain-Barre' syndrome is not known; and why the disorder only occurs in certain patients is still not known. Research to date indicates that the nerves of the GBS patient are attacked by the body's own defense system against disease-antibodies and white blood cells. As a result of this autoimmune attack, the nerve insulation (myelin) and sometimes even the covered conducting part of the nerve (axon) is damaged.

The rapid onset of (ascending) weakness, frequently accompanied by abnormal sensations and pain that affect both sides of the body similarly, is a common presenting picture, and quite often, the patient's symptoms and physical exam are sufficient to indicate the diagnosis. A lumbar puncture may be performed to find elevated protein levels in the cerebro-spinal fluid to confirm the diagnosis. The severity of Guillain-Barre' syndrome can vary greatly. In its milder form, it may cause a waddling or ducklike gait, and perhaps some tingling and upper limb weakness that may briefly, for days or weeks, impair a patients lifestyle. Some primary care physicians have described patients who complained of mild brief tingling and/or limb weakness accompanying or following a viral illness, such as a sore throat or diarrhea. Such a set of symptoms may represent a very mild form of GBS. In contrast to such mild forms, at the other extreme a GBS patient may become almost totally paralyzed and fraught with complications.

Although the exact percentages vary from study to study for long-term prognosis, up to 85 percent of GBS patients reach nearly complete recovery, although they may suffer chronic problems, such as muscular pain and weakness. Perhaps 5 to 15 percent of GBS patients will have severe long-term disabilities. Less than 5 percent die. GBS can develop in any person at any age, regardless of gender or ethnic background. Most GBS patients' health will improve significantly over time. Among GBS survivors, those patients who experienced their worst symptoms within the first seven days of the illness tend to have a worse outcome. It is important to emphasize that, as in many aspects of medicine the prognosis or expectation for degree of recovery for any particular patient cannot be predicted.

Not infrequently, after apparent recovery from Guillain-Barre' syndrome, patients may experience the recurrence of abnormal sensations, typically in the lower and/or upper limbs. They may consist of numbness, decreased sensations, tingling, burning, a sense of worms crawling under the skin, pain, muscle spasms or cramps in the form of severe Charlie Horses, and a variety of other disconcerting symptoms that the patient may even have difficulty describing.

A particularly frustrating consequence of this disorder is long-term recurrences of fatigue and/or exhaustion as well as abnormal sensations including pain and muscle aches. These problems can occur following the exertion of normal walking or working and can be alleviated by reduction of activity and rest. Many patients learn by trial and error how much activity they can tolerate.

Studies now suggest that the degree of damage or number of diminished axons, the conducting part of nerve cells or, if you will, the wire, rather than damage of its surrounding covering or insulation, the myelin sheath, may explain chronic or long-term damage and paralysis. Such studies are shedding light on some of the long-term effects of GBS. Several years after the recovery phase of the illness, some GBS patients experience symptoms identical to Post-Polio Sequela (Post-Polio Syndrome), a condition affecting many "recovered" Polio survivors.

Sometimes motor axon damage is not severe and the cells can recover much of their function. Other axons may sustain more complete and irreversible damage. Even if this is the case, however, function can often be restored by "sprouting". Motor axons have the ability to send out new branches that can innervate neighboring muscle fibers whose own axons have been destroyed. Nerve cells normally innervate between 200 and 500 individual muscle fibers. If a percentage of motor axons are destroyed, and sprouting takes place, the remaining axons may be innervating as much as four times the normal amount of muscle fiber. Some individuals may have gained a degree of recovery by building up the strength of their remaining musculature by exercise and intense use, similar to athletic training. These individuals, however, used this strength in their daily activity and thus the muscles have been performing continually at a level that is no longer tolerated.

Recurrent abnormal sensations may reflect the presence of residual nerve damage that had occurred during the initial stages of the syndrome's development. Frustration arises because the sensations are truly felt by the patient and can be quite severe or annoying but have no physical correlate outside the body and may be difficult to control. Furthermore, they can be difficult to demonstrate, measure, or otherwise document. One example is the sense of vibration while lying perfectly still in bed. Another example is the feeling of pain without an underlying injury. The pain may be severe enough so that routine analgesic medications don't give relief, and more potent analgesics such as narcotic pain relievers may be considered. The treating physician may be hard pressed to justify use of such drugs for a problem he can't prove exists. Such drugs, however, are relatively safe and there is no occurrence of addiction in these patients when given opioids to control chronic pain.

Persisting abnormal sensations, if sufficiently bothersome, may sometimes respond to a variety of treatment modalities. These can include simple and relatively safe approaches such as over-the-counter analgesics (for pain), including aspirin and acetaminophen. Some people find that the local applications of heat, especially moist heat, or cold may be beneficial. Should these initial measures give inadequate relief, alternative approaches, such as prescription medications, may be entertained, especially to treat persisting pain. For patients with persisting muscle group weakness, various methods (orthotic devices) can be used to circumvent the disability. For example, a dropped foot can be treated with a molded ankle foot orthosis (MAFO), a lightweight plastic device that fits behind the leg and under the foot.

Each case of Guillain-Barre' syndrome is different. It is important to realize that the complications and therefore treatments of Guillain-Barre' syndrome are not predictable. For the most part, treatments are highly individualized.

Articles

Residual Effects Following Guillain-Barre'

Disability After “Recovery” From GBS

What's In a Name? Important Differences
Between GBS, CIDP and Related Disorders

The Vexed Question of Residuals in GBS

Web Sites

Guillain-Barre' Syndrome Articles
http://www.angelfire.com/home/gbs/

GBS/CIDP Foundation International
http://www.gbsfi.com/

Guillain Barre' Syndrome Discussion Boards
http://www.gbsfi.com/forums/index.php

Harvest Center's Post-Polio Page
http://www.members.aol.com/harvestctr/pps/polio.html

Health, Wellness and Aging with a Disability
http://www.jik.com/hwawd.html

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