Blog > Lifestyle > Health > Guillain-Barré Syndrome, What is Guillain-Barré Syndrome?

Guillain-Barré Syndrome, What is Guillain-Barré Syndrome?

Posted on April 22, 2009

What is Guillain-Barré Syndrome?

Guillain-Barré syndrome is a disorder in which the body’s immune system attacks part of the peripheral nervous system. The first symptoms of this disorder include varying degrees of weakness or tingling sensations in the legs. In many instances, the weakness and abnormal sensations spread to the arms and upper body. These symptoms can increase in intensity until the muscles cannot be used at all and the patient is almost totally paralyzed. In these cases, the disorder is life-threatening and is considered a medical emergency. The patient is often put on a respirator to assist with breathing. Most patients, however, recover from even the most severe cases of Guillain-Barré syndrome, although some continue to have some degree of weakness. Guillain-Barré syndrome is rare. Usually Guillain-Barré occurs a few days or weeks after the patient has had symptoms of a respiratory or gastrointestinal viral infection. Occasionally, surgery or vaccinations will trigger the syndrome. The disorder can develop over the course of hours or days, or it may take up to 3 to 4 weeks. No one yet knows why Guillain-Barré strikes some people and not others or what sets the disease in motion. What scientists do know is that the body’s immune system begins to attack the body itself, causing what is known as an autoimmune disease. Guillain-Barré is called a syndrome rather than a disease because it is not clear that a specific disease-causing agent is involved. Reflexes such as knee jerks are usually lost. Because the signals traveling along the nerve are slower, a nerve conduction velocity (NCV) test can give a doctor clues to aid the diagnosis. The cerebrospinal fluid that bathes the spinal cord and brain contains more protein than usual, so a physician may decide to perform a spinal tap.

Is there any treatment?

There is no known cure for Guillain-Barré syndrome, but therapies can lessen the severity of the illness and accelerate the recovery in most patients. There are also a number of ways to treat the complications of the disease. Currently, plasmapheresis and high-dose immunoglobulin therapy are used. Plasmapheresis seems to reduce the severity and duration of the Guillain-Barré episode. In high-dose immunoglobulin therapy, doctors give intravenous injections of the proteins that in small quantities, the immune system uses naturally to attack invading organism. Investigators have found that giving high doses of these immunoglobulins, derived from a pool of thousands of normal donors, to Guillain-Barré patients can lessen the immune attack on the nervous system. The most critical part of the treatment for this syndrome consists of keeping the patient’s body functioning during recovery of the nervous system. This can sometimes require placing the patient on a respirator, a heart monitor, or other machines that assist body function.

What is the prognosis?

Guillain-Barré syndrome can be a devastating disorder because of its sudden and unexpected onset. Most people reach the stage of greatest weakness within the first 2 weeks after symptoms appear, and by the third week of the illness 90 percent of all patients are at their weakest. The recovery period may be as little as a few weeks or as long as a few years. About 30 percent of those with Guillain-Barré still have a residual weakness after 3 years. About 3 percent may suffer a relapse of muscle weakness and tingling sensations many years after the initial attack.

What research is being done?

Scientists are concentrating on finding new treatments and refining existing ones. Scientists are also looking at the workings of the immune system to find which cells are responsible for beginning and carrying out the attack on the nervous system. The fact that so many cases of Guillain-Barré begin after a viral or bacterial infection suggests that certain characteristics of some viruses and bacteria may activate the immune system inappropriately. Investigators are searching for those characteristics. Neurological scientists, immunologists, virologists, and pharmacologists are all working collaboratively to learn how to prevent this disorder and to make better therapies available when it strikes.

From Wikipedia:

Guillain-Barré syndrome
Guillain-Barré syndrome (GBS) (French pronunciation: [ɡilɛ̃ baˈʁe];[1][2] in English, pronounced /ˈɡiːlæn ˈbɑreɪ/,[3] /ɡiːˈæn bəˈreɪ/,[4] etc.[5]) is an acute inflammatory demyelinating polyneuropathy (AIDP), an autoimmune disease affecting the peripheral nervous system, usually triggered by an acute infectious process. It is included in the wider group of peripheral neuropathies. There are several types of GBS, but unless otherwise stated, GBS refers to the most common form, AIDP. It is frequently severe and usually exhibits as an ascending paralysis noted by weakness in the legs that spreads to the upper limbs and the face along with complete loss of deep tendon reflexes. With prompt treatment by plasmapheresis or intravenous immunoglobulins and supportive care, the majority of patients will regain full functional capacity. However, death may occur if severe pulmonary complications and dysautonomia are present.[6]

Pathophysiology

All forms of Guillain-Barré syndrome are due to an immune response to foreign antigens (such as infectious agents or vaccines) but mistargeted to host nerve tissues instead (a form of antigenic mimicry). The targets of such immune attack are thought to be gangliosides, which are complex glycosphingolipids present in large quantities on human nerve tissues, especially in the nodes of Ranvier. An example is the GM1 ganglioside, which can be affected in as many as 20-50% of cases, especially in those preceded by Campylobacter jejuni infections. Another example is the GQ1b ganglioside, which is the target in the Miller Fisher syndrome variant (see 2.1 below).

The most common antecedent infection is Campylobacter jejuni.[7] However, 60% of cases do not have a known cause.[8]

The end result of such autoimmune attack on the peripheral nerves is inflammation of myelin and conduction block, leading to a muscle paralysis that may be accompanied by sensory or autonomic disturbances.

However, in mild cases, axonal function remains intact and recovery can be rapid if remyelination occurs. In severe cases, such as in the AMAN or AMSAN variants (see 2.1 below), axonal degeneration occurs, and recovery depends on axonal regeneration. Recovery becomes much slower, and there is a greater degree of residual damage. Recent studies on the disease have demonstrated that approximately 80% of the patients have myelin loss, whereas, in the remaining 20%, the pathologic hallmark of the disease is indeed axon loss.

Serum sickness can rarely manifest as the Guillain-Barre syndrome (GBS), one of its most serious complications. The most serious complications of serum sickness are nerve conditions and peripheral neuritis. Serum sickness is of itself a type of delayed allergic response.

GBS is a form of autoimmune disease with a delayed hypersensitivity reaction, or a rare manifestation of serum sickness, or transient syndrome resembling serum sickness with loss of appetite, nausea, vomiting, and stomach pain accompanied by weakness (tired feeling), chills, low grade fever and possible evidence of brain involvement, indicated by lethargy and migraine headaches, although one theory of the cause of migraine is a central nervous system (CNS) disorder, or Bickerstaff’s brain stem encephalitis, a regional variant of GBS. Typical pain is occipital or in the back of the head. Alterations of consciousness go with this headache type affecting the brainstem implicated in the maintenance of arousal, but is a worrisome feature of this type of headache called a Bickerstaff migraine. Other features of Bickerstaff migraine go with involvement primarily of the brainstem including clumsiness and gait unsteadiness, ie. “pulling to the right”..

Categories: Health

Leave Comment

(required)

(required)