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Alternative Names: Acute Inflammatory Demyelinating Polyneuropathy or Landry's Ascending Paralysis.
Guillain-Barré syndrome (GBS) is an inflammatory disorder of the peripheral nerves, which are those outside the brain and spinal cord. It is a neurological disorder in which the body's immune system attacks part of the peripheral nervous system, devastating because of its sudden and unexpected onset.
Normally, the cells of the immune system attack only foreign material and invading organisms, but in Guillain-Barre syndrome, the immune system starts to destroy the myelin sheath that surrounds the axons of many nerve cells, and sometimes the axons themselves.
The myelin sheath surrounding the axon speeds up the transmission of nerve signals and allows the transmission of signals over long distances. When they are injured or degraded, nerves cannot send signals efficiently, and muscles begin to lose their ability to respond to the brain's commands. And, the brain also receives fewer sensory signals from the rest of the body, resulting in an inability to feel textures, heat, pain, and other sensations.
If Guillain-Barre is preceded by a viral infection, it is possible that the virus changes the nature of cells in the nervous system causing the immune system to interpret them as foreign cells. It is also possible that the virus makes the immune system itself less discriminating about what cells it attacks.
Incidence; Causes & Development
Afflicting about one person in 100,000, GBS can develop in any person at any age, regardless of gender or ethnic background.
The cause of GBS is not known. Perhaps 50% of cases occur shortly after a microbial (viral or bacterial) infection such as a sore throat or diarrhea. Many cases developed in people who received the 1976 swine flu vaccine. Some theories suggest an autoimmune mechanism, in which the patient's defense system of antibodies and white blood cells are triggered into damaging the nerve covering or insulation, leading to weakness and abnormal sensation.
Signs & Symptoms
The disorder can develop over the course of hours or days, or it may take up to three to four weeks. GBS is characterized by the rapid onset of weakness and often paralysis of the legs, arms, breathing muscles and face. Abnormal sensations often accompany the weakness.
The symptoms of GBS vary a great deal from patient to patient, and so each has a unique case history. Pain appears to be a common feature and the symptoms usually appear soon after an infection. They increase in intensity over a period of time, varying from a few hours in serious cases, to around 4 weeks in most patients. The symptoms vary, also with regard to the pattern in which they appear. Their distribution is usually symmetrical and double-sided. This means that the symptoms appear on both the left and right side of the body, but they may also appear randomly, especially in the beginning. They may appear for a short while, disappear for some days, and reappear shortly.
GBS symptoms vary, depending on whether the syndrome has affected the sensory nerve fibres (that transmit signals from a body area to the brain and spine), or the motor nerve fibres (that transmit signals from the brain and spine to a body area). When both motor and sensory nerves are affected, the patient experiences a mixture of symptoms. The patient may also experience disruption in the working of the autonomous nervous system.
The combination of pain, weakness and sensory disruptions are generally so alarming that the more discreet changes in the patient's autonomous nerve system may be overlooked. These may be just as serious, however.
The few patients who experience a mild attack of GBS feel weak, may think they have a viral infection and perhaps experience a brief sensory reduction. They are not ill enough to seek a doctor, and as the illness disappears completely after a few days or weeks, they may be completely ignorant of the fact that they've just had GBS.
The stage of greatest weakness is usually within the first two weeks after symptoms appear, and by the third week of the illness 90% of all patients are at their weakest.
In moderate cases, the patient may partially or completely lose the ability to walk and carry out daily chores. This frightens the patient, who then seeks medical help after a while. The course of the syndrome is unpredictable, and the patient is generally hospitalised immediately for diagnosis, observation and treatment.
Unfortunately, the majority of patients experience GBS as the frightening illness it is. Patients may be almost completely paralysed for a while.
In very serious cases, the entire body can be paralysed, even including the eye muscles. GBS can - very rarely - present with coma and absent brainstem reflexes. Such patients are NOT brain dead; their brains and sense of hearing and smell work perfectly well, and the patient is alert and conscious of what is going on around him or her. But he or she can literally not move a muscle in response.
In some cases, the face may be affected when damage occurs to the cranial nerves. These connect the brain to the muscles of the jaw, face and tongue, and also control the muscles that move the patient's head, neck and shoulders. As the paralysis progresses, all these areas may be paralysed. The eyelids or one side of the face may droop, the face loses it's ability to express feelings.
The autonomous nerves control the inner organs, whose function is normally carried out automatically, such as secretion of hormones, vision, urination, breathing, heart beat, etc. These functions may be disrupted, resulting in arrhythmia, unstable blood pressure, blurred or double vision, dizziness, fainting spells, inability to regulate the body temperature, difficulty breathing, reduced ability to control the function of the stomach, digestive system and bladder, loss of weight, vomiting after meals, reduced function of various glands, incontinence, impotency, etc. It may be difficult to urinate, and the bladder may feel as it is not being emptied.
Diagnosis & Tests
Guillain-Barre is called a syndrome rather than a disease because it is not clear that a specific disease-causing agent is involved. The signs and symptoms of the syndrome can be quite varied, so doctors may, on rare occasions, find it difficult to diagnose Guillain-Barre in its earliest stages.
In addition, other disorders have symptoms similar to those found in Guillain-Barre, so doctors evaluate carefully before making a diagnosis. Signs and symptoms that may differentiate Guillain-Barre from other disorders include the following. With Guillain-Barre:- symptoms appear on both sides of the body
- symptoms appear quickly -- days or weeks as opposed to months
- reflexes are usually lost
- cerebrospinal fluid that bathes the spinal cord and brain contains more protein than usual.
Quite often, the patient's symptoms and physical exam are sufficient to indicate the diagnosis. To confirm the diagnosis, a lumbar puncture to find elevated fluid protein and electrical test of nerve and muscle function may be performed.
Treatment & Prevention
Currently, there is no known cure for Guillain-Barre syndrome. However, there are therapies that lessen the severity of the illness, as well as ways to treat symptoms and complications of the disorder.
Many patients require an intensive care unit during the early course of their illness, especially if support of breathing with a machine is required.
Because progression of the disease in its early stages is unpredictable, most newly diagnosed patients are hospitalized and usually placed in an intensive care unit to monitor breathing and other body functions.
Care involves use of general supportive measures for the paralyzed patient, and also methods specifically designed to speed recovery, especially for those patients with major problems, such as inability to walk. Plasma exchange (a blood "cleansing" procedure) and high dose intravenous immune globulins are often helpful to shorten the course of GBS.
Specific treatment for Guillain-Barre syndrome will be determined by physician(s) based on:- patient's overall health and medical history
- extent of the disease
- patient's tolerance for specific medications, procedures, or therapies
- expectations for the course of the disease
- patient's opinion or preference.
Plasmapheresis and high-dose immunoglobulin therapy may be used in some cases of Guillain-Barre syndrome. Treatment for the syndrome mainly consists of keeping the patient's body functioning during recovery of the nervous system. This may require placing the patient on a respirator, heart machine, or other machines that assist body function.
Even before recovery begins, caregivers begin to manually move the patient's limbs to help keep the muscles flexible and strong. As soon as the patient begins to recover, physical therapy begins.
The need for sophisticated technology and close observation for complications in the paralyzed patients are reasons that Guillain-Barre syndrome is treated in hospitals.
Prognosis; Complications; Seek medical attention if
Although most people recover, this can take months, and some may have long term disabilities of varying degrees. Fewer than 5% die.
The recovery period may be as little as a few weeks or as long as a few years. About 30% of patients still feel a residual weakness after three years, and 3 to 5% may suffer relapse of muscle weakness and tingling sensations many years after the initial attack.
GBS can be more serious in certain parts of the body than in others, and even life-threatening. The progressive paralysis displays varying intensity: It can stop suddenly, causing partial paralysis, or may continue until the entire body is paralysed.
If the paralysis affects the chest muscles, they are weakened or paralysed, affecting the patient's breathing and heart. Heart failure may occur due to strain.
Symptoms to watch for include those that indicate sensory and/or motor nerves are affected:- Muscle weakness or paralysis that begins in the feet or legs and gradually spreads to the arms and body, or vice-versa, from the arms and moving downward
- Lack of coordination
- Sensory disturbances, muscular pain.
Symptoms to be especially watchful for are those that indicate that the autonomous nerves are affected, and that vital body functions are affected:- Blurred vision
- Difficulty moving the facial muscles
- Dizziness
- Irregular heart beat.
If these symptoms are seen, contact a doctor as soon as possible.
Symptoms of possible life-threatening situations include:- Difficulty breathing, breathlessness, difficulty taking deep breaths
- Drooling, difficulty swallowing
- Fainting spells.
If the patient experiences any of these symptoms, medical help should be sought immediately.
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Signs, symptoms & indicators of Guillain-Barre Syndrome: | | | | | Minor Symptoms |  Lack of/weak tendon reflexes | Loss of reflexes, such as the knee jerk, are usually found. |
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Symptoms - Aging |
Decreased ability to walk| | Only about 28% of patients with the GBS remain able to walk unaided. Patients with the hyper acute form of the syndrome lose the use of their legs within a day. |
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Symptoms - General |
 Fatigue of recent onset| | The rapid onset of (ascending) weakness, frequently accompanied by abnormal sensations that affect both sides of the body similarly, is a common presenting picture. |
Poor bodily coordination| | The patient may have difficulty holding and manipulating objects, such as buttons and toothbrushes. |
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Symptoms - Head - Mouth/Oral |
Voice change| | The patient's voice may change because the vocal chords are affected. |
Incoherent speech| | Speech may be unintelligible, because the various muscles required to form speech are weakened. |
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Symptoms - Head - Nose |
Reduced sense of taste or smell |
Symptoms - Metabolic |
Feeling unusually cold |
Symptoms - Muscular |
Individual weak muscles| | GBS can cause muscles to weaken and atrophy. Progressive weakening or paralysis may occur, typically beginning in the feet, hands or face. The paralysis characteristically involves more than one limb, most commonly the legs. The paralysis is progressive and usually ascending, spreading to the rest of the limb, and from there may spread to the legs, arms and the rest of the body. The arms may feel weak, the patient no longer being able to lift heavy objects. |
Heaviness of the legs |
Symptoms - Nervous |
Not feeling injuries
Being over-sensitive to touch| | In some cases, pain may be so severe so as to limit walking. Patients with symptoms limited to the feet and ankles may observe similar symptoms in the fingertips; as the symptoms extend to the knees, they may also extend to the wrists. Only rarely do these symptoms extend above the knees. |
Numb/tingling/burning extremities| | Symptoms generally begin in the patient's feet, hands or face, spread to the legs or arms, and increase in intensity as they move towards the center of the body. They generally appear on both left and right sides of the body. However, GBS is unpredictable, and cases have been reported in which this "glove and stocking" pattern is not followed. Instead, motor symptoms or disruptions in the autonomous system may be observed. GBS may also affect an arm or a leg alone, without spreading to the rest of the body. |
(Recent) loss of sense of touch| | The sensory symptoms make themselves felt as the sensory nerves are attacked. The patient experiences loss or reduction of the sense of touch, or abnormal sensations such as burning, tingling, pins and needles, 'ants under the skin', vibrations, numbness, etc. |
Inablility to distinguish hot & cold |
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Conditions that suggest Guillain-Barre Syndrome: | | | | | Aging | Hearing Loss| | Deafness is unusual but has been reported. |
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Digestion |
Constipation| | Constipation is often a problem, due to the reduced activity of the intestines, change of diet, weakened stomach muscles that resist efforts by the patient to express the intestinal content. |
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Musculo-Skeletal |
Muscle Pains (Myalgia)| | Gradually muscle pain is experienced in the large muscles, such as the thighs, back and shoulders. Pain in the lower back, buttocks or thighs is common, and is often the earliest symptom. Deep, aching muscle pain is common. |
Muscle Cramps / Twitching| | Stiffness and cramping pain is common. Weakness caused by GBS may be accompanied by pain and muscle spasms. |
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Pain |
Low Back Pain| | Pain in the lower back, buttocks or thighs is common, and is often the earliest symptom. |
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Skin-Hair-Nails |
Excessive Sweating |
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Guillain-Barre Syndrome can lead to: | | | | | Musculo-Skeletal | Muscle Pains (Myalgia)| | Gradually muscle pain is experienced in the large muscles, such as the thighs, back and shoulders. Pain in the lower back, buttocks or thighs is common, and is often the earliest symptom. Deep, aching muscle pain is common. |
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Pain |
Low Back Pain| | Pain in the lower back, buttocks or thighs is common, and is often the earliest symptom. |
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Guillain-Barre Syndrome could instead be: | | | | | Infections | Lyme Disease| | Putting aside the issue of whether Lyme disease does or does not cause some cases of Guillain-Barre syndrome, the two diseases share so many symptoms that Lyme disease can be mistaken for Guillain-Barre syndrome. |
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Recommendations and treatments for Guillain-Barre Syndrome: | | | | | Physical Medicine |  Physical Therapy| | Most patients, after an early hospital stay and when medically stable, are candidates for a rehabilitation program to help learn optimal use of muscles as nerve supply returns. |
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KEY | | Weak or unproven link | | | Strong or generally accepted link | | | Highly recommended |
GLOSSARY
Acute
An illness or symptom of sudden onset, which generally has a short duration.
Antibody (Antibodies)
A type of serum protein (globulin) synthesized by white blood cells of the lymphoid type in response to an antigenic (foreign substance) stimulus. Antibodies are complex substances formed to neutralize or destroy these antigens in the blood. Antibody activity normally fights infection but can be damaging in allergies and a group of diseases that are called autoimmune diseases.
Arrhythmia (Arrhythmias)
A condition caused by variation in the regular rhythm of the heartbeat. Arrhythmias may cause serious conditions such as shock and congestive heart failure, or even death.
Autoimmune Disease (Autoimmune, Autoimmunity)
One of a large group of diseases in which the immune system turns against the body's own cells, tissues and organs, leading to chronic and often deadly conditions. Examples include multiple sclerosis, rheumatoid arthritis, systemic lupus, Bright's disease and diabetes.
Axon
An extended process of a neuron that conducts impulses traveling away from the cell body.
Bacteria (Bacterial, Bacterium)
Microscopic germs. Some bacteria are "harmful" and can cause disease, while other "friendly" bacteria protect the body from harmful invading organisms.
Constipation (Constipated)
Difficult, incomplete, or infrequent evacuation of dry, hardened feces from the bowels.
Cramp (Cramping, Cramps)
A sudden, involuntary, painful muscular contraction.
Diarrhea
Excessive discharge of contents of bowel.
Gland (Glands)
The glandular system is one of the most important and complicated systems of the body. Gland tissue can be either an organ or general tissue that secretes chemicals and there are two types of gland: exocrine and endocrine. Those glands which secrete chemicals through tubules or ducts are called exocrine and include sweat, tear and salivary glands. Ductless glands - part of the endocrine system - secrete special chemicals (hormones) directly into the blood.
Hormones (Hormone)
Chemical substances secreted by a variety of body organs that are carried by the bloodstream and usually influence cells some distance from the source of production. Hormones signal certain enzymes to perform their functions and, in this way, regulate such body functions as blood sugar levels, insulin levels, the menstrual cycle, and growth. These can be prescription, over-the-counter, synthetic or natural agents. Examples include adrenal hormones such as corticosteroids and aldosterone; glucagon, growth hormone, insulin, testosterone, estrogens, progestins, progesterone, DHEA, melatonin, and thyroid hormones such as thyroxine and calcitonin.
Immune System (Immune Response, Immunity)
A complex that protects the body from disease organisms and other foreign bodies. The system includes the humoral immune response and the cell-mediated response. The immune system also protects the body from invasion by making local barriers and inflammation. The process may involve acquired immunity (the ability to learn and remember a specific infectious agent), or innate immunity (the genetically programmed system of responses that attack, digest, remove, and initiate inflammation and tissue healing).
Incontinence (Incontinent)
The inability to retain urine in the bladder for a reasonable length of time. It is can be caused by urethral irritation, loss of tone to the basement muscle of the bladder (the trigone), scarification or growths on the urethral lining, nerve damage, or emotional stress.
Myelin
A substance made of protein and lipid (fat) that protects the nerves, especially in the brain. The myelin sheath is a jacket of insulation around axons to help them conduct their electrical discharges quickly down the axon.
Nervous System
A system in the body that is comprised of the brain, spinal cord, nerves, ganglia and parts of the receptor organs that receive and interpret stimuli and transmit impulses to effector organs.
Protein (Proteins)
Compounds composed of hydrogen, oxygen, and nitrogen present in the body and in foods that form complex combinations of amino acids. Protein is essential for life and is used for growth and repair. Foods that supply the body with protein include animal products, grains, legumes, and vegetables. Proteins from animal sources contain the essential amino acids. Proteins are changed to amino acids in the body.
Spinal Tap (Lumbar Puncture)
Despite the discomfort felt by many over this procedure, spinal taps are actually very simple procedures from the doctor's standpoint. Because the fluid that coats and flows through the brain also flows down into the spinal cord, the spinal fluid reflects what is occurring in the brain. A spinal tap involves: numbing the skin over the lower part of the spine with an injection or cream; sterilizing the area; having the patient curl up on his side while being held securely; passing a needle through the skin at a large space in between two of the lower vertebrae (the needle does NOT pass through bone); slowly advancing the needle until it "pops" through the lining of the spinal canal into an area of spinal fluid (the needle does NOT go into the spinal cord where the nerves are because the spinal cord stops further up the spine: the bottom of the spine simply contains a reservoir of spinal fluid); spinal fluid will flow out through the needle and the doctor will collect it and send it to the lab. Once the needle is pulled out, the hole seals up rapidly.
Stomach
A hollow, muscular, J-shaped pouch located in the upper part of the abdomen to the left of the midline. The upper end (fundus) is large and dome-shaped; the area just below the fundus is called the body of the stomach. The fundus and the body are often referred to as the cardiac portion of the stomach. The lower (pyloric) portion curves downward and to the right and includes the antrum and the pylorus. The function of the stomach is to begin digestion by physically breaking down food received from the esophagus. The tissues of the stomach wall are composed of three types of muscle fibers: circular, longitudinal and oblique. These fibers create structural elasticity and contractibility, both of which are needed for digestion. The stomach mucosa contains cells which secrete hydrochloric acid and this in turn activates the other gastric enzymes pepsin and rennin. To protect itself from being destroyed by its own enzymes, the stomach’s mucous lining must constantly regenerate itself.
Syndrome
A medical condition characterized by a collection of related symptoms (what the patient feels) and signs (what a doctor can observe or measure).
Virus (Viri, Viruses)
Any of a vast group of minute structures composed of a protein coat and a core of DNA and/or RNA that reproduces in the cells of the infected host. Capable of infecting all animals and plants, causing devastating disease in immunocompromised individuals. Viruses are not affected by antibiotics, and are completely dependent upon the cells of the infected host for the ability to reproduce.
White Blood Cell (WBC, White Blood Cells)
A blood cell that does not contain hemoglobin: a blood corpuscle responsible for maintaining the body's immune surveillance system against invasion by foreign substances such as viruses or bacteria. White cells become specifically programmed against foreign invaders and work to inactivate and rid the body of a foreign substance. White blood cells are composed primarily of neutrophils, monocytes and lymphocytes. Lymphocytes are either T-cells or B-cells. T-cells (CD3 cells) are divided into T-helper (CD4 cells) and T-suppressor/cytotoxic (CD8 cells) cells.
Last updated: Apr 13, 2008
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