Guillain-Barre Syndrome

Guillain-Barre Syndrome: Overview

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.

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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 and 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 and 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 fibers (that transmit signals from a body area to the brain and spine), or the motor nerve fibers (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 hospitalized immediately for diagnosis, observation and treatment.

Unfortunately, the majority of patients experience GBS as the frightening illness it is.  Patients may be almost completely paralyzed for a while.

In very serious cases, the entire body can be paralyzed, 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 paralyzed.  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 and 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 and 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 paralyzed.

If the paralysis affects the chest muscles, they are weakened or paralyzed, 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:

If the patient experiences any of these symptoms, medical help should be sought immediately.

Signs, symptoms & indicators of Guillain-Barre Syndrome:

Lab Values - Cells

Symptoms - General

Recent onset fatigue

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.

Symptoms - Head - Mouth/Oral

(Slight/major) 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.

(Much) reduced sense of taste

The sense of taste may become altered or reduced.

Symptoms - Head - Nose

Symptoms - Metabolic

Symptoms - Muscular

Specific/severe muscle weakness

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.

Symptoms - Nervous

Increased/extreme sensitivity 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.

(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.

Numb/burning/tingling 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.

Symptoms - Skeletal

(Slightly/severely) impaired ability to walk or inability 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.

Conditions that suggest Guillain-Barre Syndrome:

Aging

Hearing Loss

Deafness is unusual but has been reported.

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.

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.

Pain

Low Back Pain

Pain in the lower back, buttocks or thighs is common, and is often the earliest symptom.

Skin-Hair-Nails

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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.

Pain

Low Back Pain

Pain in the lower back, buttocks or thighs is common, and is often the earliest symptom.

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.

Recommendations 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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