An Overview of Guillain Barré Syndrome
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Acknowledgments
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"An
Overview for the LAY PERSON" was written by Dr. Joel
S. Steinberg, Neurologist, and a former GBS patient. This
booklet is dedicated to founding members Estelle and Robert
Benson of: The Guillain-Barré Syndrome Foundation International
PO BOX 262 WYNNEWOOD
GBS Tasmania wishes to thank the Guillain-Barré Syndrome Foundation
International for the use of their material.
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INTRODUCTION
The disorder commonly called Guillain-Barré (ghee-yan bar-ray)
syndrome is a rare illness that affects the peripheral nerves of
the body. It can cause weakness and paralysis, as well as abnormal
sensations. The syndrome occurs sporadically, that is, it cannot
be predicted, and can occur at any age and to both sexes. It can
vary greatly in severity from the mildest case, that may not even
be brought to the doctors' attention, to a devastating illness with
almost complete paralysis that brings a patient close to death.
Because it is so rare, most of the public has never heard of the
illness, or if they did, know little about it. Yet, for those effected,
the illness can be severely disabling.
The following Overview is directed to patients with Guillain-Barré
syndrome, their families and other interested lay persons. Its
aim is to briefly acquaint you with the illness' history, its
cause and manner of presentation, describe some of the effects
of this disorder on the patient's life and those about him or
her.
The term syndrome, rather than disease, is used to describe
the illness observed by Guillain and others. This term reflects
the recognition of the illness by the collection of symptoms (what
the patient tells the physician about changes in his body) that
typify the disorder.
HISTORICAL BACKGROUND
In 1859, a French physician, Jean B.O. Landry, described in detail
a disorder of the nerves that paralysed the legs, arms, neck and
breathing muscles of the chest. Several reports of a similar disorder
followed from other countries. The demonstration by Quinke in 1891
of spinal fluid removed by passing a needle into the lower back
paved the way for three Parisian physicians, Georges Guillain, Jean
Alexander Barré and Andre Strohl to show, in 1916, the characteristic
abnormality of increased fluid protein, but normal cell count. Since
then, several investigators have collected additional information
about this disorder. It can affect nerves not only to the limbs
and breathing muscles, but also those to the throat, heart, urinary
bladder and eyes. Doctors have several names for the syndrome, including
acute (rapid onset of), idiopathic (of unknown cause), polyneuritis
(irritation or inflammation of many nerves), acute idiopathic polyradiculoneuritis,
Landry's ascending paralysis, etc.
CAUSES OF GUILLAIN-BARRÉ SYNDROME
The cause of Guillain-Barré syndrome is not known. A variety
of events seem to trigger the illness. Many cases occur a few
days to a few weeks after a viral infection. These infections
include the common cold, sore throat, and stomach and intestinal
viruses, with vomiting and diarrhoea. Some cases have been associated
with specific viral infections, such as infectious mononucleosis
and viral hepatitis; others have occurred with a rare disease
of red blood cells, porphyria. Some cases have occurred after
such seemingly unrelated events such as surgery, insect stings
and various injections. In the USA many cases occurred in the
winter of 1976-77 in persons who received the swine flu' vaccine.
Although many illnesses or other events seem to trigger Guillain-Barré
syndrome, why the disorder occurs in certain patients is still
not known.
Research to date indicates that, regardless of the triggering event,
the nerves of the Guillain-Barré patient are attacked by the body's
defence system against disease-antibodies and white blood cells.
As a result of this attack, the nerve insulation (myelin) and sometimes
even the covered conducting part of the nerve (axon) is damaged
and signals are delayed or otherwise changed. Abnormal sensations
and weakness follow.
Because Guillain-Barré syndrome often follows a viral illness,
it is sometimes mistakenly thought to be contagious. However, there
is no evidence that it can be caught even if a person was around
the patient when they had the preceding viral infection. In fact,
often the virus is no longer in the patient when they develop the
syndrome.
EARLY FINDINGS WITH GUILLAIN-BARRÉ SYNDROME
The effects of Guillain-Barré syndrome can be quite varied. As
mentioned above, this disorder affects the nerves that sense our
surroundings (hot, cold, smooth, rough, other textures etc.) as
well as those that signal our muscles to contract and let us walk,
write, breathe, swallow, talk, smile, and so forth. In Guillain-Barré
syndrome, the damaged nerves can not perform these functions properly.
If nerves that sense surroundings become damaged, patients may
initially develop abnormal or decreased feelings, such as numbness,
tingling, asleep feeling, crawling under the skin, electricity,
vibrations and so forth. These abnormal sensations are often felt
in the feet, hands, and even gums or face, tend to be equal on both
sides of the body, and may go up the body (ascend) from feet to
hands to face, or descend.
Just as often, and usually of most distress to the patient, is
muscle weakness and aches. These are caused by damage to the nerves
that go to muscles. Commonly, the thigh and hip muscles are affected
so that walking stairs or getting up from a chair becomes difficult.
Indeed this is a problem that often motivates a patient to seek
medical attention. If the arms are affected, lifting heavy objects
becomes difficult. Aches or cramps often accompany muscle weakness.
On occasion, the muscles that control breathing lose their signals,
so a patient feels short of breath; or muscles for swallowing become
weak so that patients cough on their own secretions. Should facial
muscles lose their signals, the expression may become lop-sided,
the smile lost, or food may get caught in a cheek pouch. Such problems
may lead a person to seek medical attention. Rarely, difficulty
in urinating or inability to hold one's urine, and therefore wetting,
may be a patient's initial problem.
The syndrome may also involve the automatic nerves to the body
and alter blood pressure, heart rate, body temperature and vision.
Even brain control of kidneys can be affected, resulting in poor
or excessive urine output and abnormal blood chemistries, such as
low salt concentration.
In contrast to a stroke that typically affects only one side of
the body, Guillain-Barré syndrome usually causes sensations and
weakness of both sides.
DIAGNOSIS
As might be gathered from the above descriptions, the initial and
subsequent abnormalities in Guillain-Barré syndrome can be quite
varied. Furthermore, the symptoms can occur quite rapidly, over
hours to days, or slowly, over weeks. Thus, the diagnosis of Guillain-Barré
syndrome can be difficult to make, especially in its early phase.
Sometimes a person may have only developed abnormal sensations,
such as tingling in the hands, feet or face or easy fatigue, when
they first seek a physician's advice. In this early phase, objective
evidence of a problem may be difficult to find, and the possibility
of an emotional disturbance, rather than the presence of a true
organic illness may be suspected.
In many patients, the typically described rapid onset of equal
weakness or paralysis of both legs, and then arms, occurs, as well
as numbness, or a sense of pins and needles, and the diagnosis is
more rapidly made. As a general rule, if the onset of symptoms is
rapid and equal on both sides of the body, the syndrome is easier
to diagnose.
A variety of findings on physical examination, as well as laboratory
studies help the physician establish the diagnosis. Muscle strength
testing shows weakness; tests of sensation reveal deficits. Reflexes,
such as knee jerks, are usually lost. Conduction of electric signals
by nerves become prolonged or slow and muscle responses to nerve
stimulation becomes abnormal. Physicians may use instruments with
needles to detect these abnormalities. Also, the fluid bathing the
spinal cord in the back is usually abnormal, so that a spinal tap
is helpful. (In spite of experiences in prior years, headaches are
rare after this uncomfortable, but rarely painful, procedure.)
A diagnosis of Guillain-Barré syndrome is usually based upon evaluating
many of the findings described above. No one observation alone is
usually sufficient to make the diagnosis.
EARLY HOSPITAL STAY AND CARE
When a diagnosis of Guillain-Barré syndrome is made, most patients
are hospitalised if they are not already in such a facility. In
some patients the course is often not predictable and may be downhill.
Since important bodily functions, such as breathing, blood pressure,
heart rate, swallowing, air clearance and bladder control can
be affected, careful observation in the hospital, often in the
intensive care unit, is utilised. There, rapid treatment can be
given if problems arise.
Several doctors may be involved in early care. These may include
the family GP, the general physician, or a neurologist.
Sometimes a specialist in rehabilitation medicine is involved.
In the early stages of a severe case nursing care is very important.
On-going treatment by a physiotherapist is usually employed.
During the early part of the illness, especially for the few patients
who require intensive care, events can be quite frightening. Most
patients with Guillain-Barré syndrome were formerly healthy, so
that finding themselves suddenly paralysed, helpless, with intravenous
lines, a bladder catheter, and a heart beat monitor that continuously
beeps can be emotionally upsetting. If the arms are too weak, even
brushing teeth, feeding oneself or scratching an itch can become
very frustrating. The feeling of utter helplessness, and hopelessness,
thoughts of possible death, and the threat of permanent disability,
dependence and income loss can be emotionally overwhelming. It is
helpful for both patient and family to keep in mind that most Guillain-Barré
syndrome patients get better, most eventually walk, and many can
ultimately resume a normal life.
EMOTIONAL REACTIONS
Patients may go through a variety of emotional reactions to the
weakness, unpredictability and other aspects of GBS, including;
denial, shock and disbelief (this can't be happening to me, I must
have a more common, treatable disease, etc.,); bargaining (if I
get better fast or off the respirator or really have a more benign
disease, in return I'll...... be satisfied, do the following, etc.,);
frustration (I'm fed up with being in the hospital and want to be
back home, I'm tired of needing to wait for others to help me, be
dependent on others); depression (I feel terrible, will I never
get better, deserve this punishment, am worn out, can't put up with
this any longer) and acceptance (I'll do the best I can, things
could be worse, thank goodness I'm alive, am finally walking with
only a cane, without a cane, etc.)
Guillain-Barré syndrome patients, especially those in an intensive
care unit or on a respirator (breathing machine) may benefit emotionally
and otherwise from the following suggestions for hospital staff
and family.
Early in the hospital stay the patient may benefit from an explanation
of the disease, and the relatively good chance for recovery. If
family, friends and medical personnel also understand the illness,
they can present a more optimistic attune to the patient.
The patient on a respirator may feel less frustrated if a method
to communicate with others is provided.
It is helpful to have a central figure upon whom the patient and
family can call to get explanations of the patient's status and
care plans. Optimally, this should be an accessible person with
good bedside rapport. In the acute hospital it is often the neurologist
or general physician although in the rehabilitation centre later
the rehabilitation specialist may be the person.
Explanations of their activities by nurses, respiratory therapists,
physical therapists, etc., will help alleviate anxiety when unfamiliar
procedures are performed.
Frequent visiting by family and friends will show caring and provide
moral support.
Especially in windowless intensive care units, a clock, electric
calendar and radio will help the patient keep track of day and night
hours, maintain awareness of the outside world, improve orientation
and minimise confusion.
Should abnormal sensations develop, the patient may be relieved
to know that these are common and can often be controlled.
Allowing the patient to ventilate emotional reactions such as anger,
frustration and fear will help them better deal with these.
Involvement by family and friends at the bedside with such activities
as grooming, reading, cards, discussing family events and so forth
will reduce the patient's sense of isolation and helplessness that
a prolonged hospital stay can foster.
EARLY TREATMENT
As of this writing, specific treatments that might predictably halt
or reverse the disorder have not been definitely established although
plasmapheresis now appears to have a definite role in some cases.
Two avenues of therapy have been tried, corticosteroids (kor-ti-co-steer-oids)
and plasmapheresis (plaz-ma-fer-eace-is).
High doses of corticosteroids, hormones normally made by adrenal
gland (above the kidney) have been used with varying results. Their
value is still questionable. A large study conducted in England
indicated that corticosteroids are not beneficial.
Plasmapheresis, a process in which some of the patient's blood
is removed, the liquid part separated, and the blood cells returned
to the body, has been used for severe cases. The co-operative study
undertaken in the US seems to indicate that patients treated early
in their illness generally will recover more rapidly than those
untreated but other factors including the age of the patient and
accessibility of plasmapheresis need to be considered.
Most other treatments are directed at preventing or treating the
complications of Guillain-Barré syndrome. For example, the paralysed
patient, at bed rest, is prone to several problems that can be prevented.
Frequent turning, a foam mattress cover or special bed that enables
changes of body position, helps to prevent bedsores (skin breakdown
over bony prominences). Blood flow tends to be slow in the leg and
pelvic veins of paralysed patients and the use of blood thinners
helps to prevent formation of clots in veins and their travel to
the lungs. Leg swelling, related to paralysis and fluid accumulation
can be relieved by leg elevation, special (TED) stockings and other
techniques.
Should abnormalities of the body's internal organs develop, appropriate
treatments are available. Constipation can be relieved with bowel
softeners or other drugs. Abnormal blood pressure or heart beat
can also be treated by several medications. Retention of urine may
require bladder drainage with a catheter, called a Foley.
If muscles used for breathing become too weak, a tube is passed
into the airway (trachea), and connected to a breathing machine
or mechanical ventilator (eg. Bennet MA-1). Should these steps be
required, various methods are used to clear the lungs of secretions
and help prevent pneumonia. Because patients on a respirator cannot
speak, they may need alternate methods to communicate with hospital
personnel and family. If the hands are strong enough, a pencil and
paper on a clipboard can be used. The extremely weak patient can
be instructed to use a simple code system such as eye blinks or
finger taps to signal "yes" or "no" responses.
Even the weakest patient can usually still hear quite well, even
if completely paralysed. Thus they may still benefit from words
of encouragement and explanation of activities around them. Mechanical
support of breathing is continued until sufficient strength of required
muscles has returned. Various methods are used to determine that
strength is adequate to allow unassisted breathing and weaning from
a respirator.
During the early part of the acute hospital stay, physical therapy
is usually started even if the weakness is negligible. In fact,
this is a most important part of the patient's care. Such treatment
may include movement by a therapist of limbs to prevent abnormal
bending of joints (contractures) as well as bedsores. Special attention
is usually given to the knee, ankle, hip, shoulder, elbow and wrist
joints. A footboard or other device may be used to prevent foot
drop.
Pain of joints and muscles can be treated with pain medications
(analgesics), such as aspirin, or stronger prescription agents,
if needed. Muscle spasms can be treated with relaxants, such as
diazepam (Valium®).
During the syndrome's earliest stages, and even through its entire
course, the patient may experience difficulty and frustration from
abnormal sensations. The frustration arises because the sensations
are truly felt by the patient and can be quite severe or annoying,
but have no physical correlation outside the body (eg. the feeling
of pain, but without injury) and may be difficult to control.
Furthermore, they can be quite difficult to demonstrate, measure,
or otherwise document. One example is the sense of vibration while
lying perfectly still in bed, a phenomenon experienced by the author.
Another example is the sense of excruciating pain, so severe that
analgesic medications don't give relief and potentially addicting
narcotics may be considered. The treating physician may be hard
pressed to justify use of such drugs for a problem he can't prove
exists. Other sensation abnormalities may be quite subtle and difficult
for even the patient to describe. The author, for example, would
cough, choke and aspirate the ice cold water used to take medications.
Room temperature water was easily tolerated. This problem probably
reflected, in part, decreased sensation by cold receptors in the
throat.
Most of the various sensation problems usually resolve with time.
Particularly bothersome sensations may sometimes respond to treatment
with such drugs as phenytion (Dilantin®) or carbamazeprine (Tegretol®).
As these agents can have significant side effects, the decision
to use them should take into consideration the potential benefits
versus risks. Some-times, pain can be relieved by a transcutaneous
electric nerve stimulator (TENS) unit, a portable, battery powered
device that supplies electric current to the skin and underlying
nerves. It is important to realise that the complications and therefore
treatments of Guillain-Barré syndrome are not predictable. For the
most part, treatments are highly individualised.
INTERMEDIATE COURSE AND REHABILITATION
The downhill course of Guillain-Barré syndrome can vary from a few
days to a few weeks. Usually, a low stable level of impairment (paralysis,
weakness etc.) is then maintained for a variable length of time
- days to weeks, and rarely, longer. However, once improvement begins,
relapse or recurrence of the syndrome is rare. Generally, the shortest
the time for recovery to begin after maximum disability is reached,
the less likely that some long-term disability will remain.
When the patient has recovered from medical com-plications, such
as breathing difficulty and infections, and some muscle strength
has returned, treatment in an acute care hospital is usually no
longer required. Physical therapy is continued and if the patient
is sufficiently weak they are transferred to a rehabilitation hospital.
In the rehabilitation centre, emphasis is placed on regaining maximal
use of the weak muscles. Simultaneously, any remaining medical complications
are treated. These can include control of high blood pressure, antibiotics
for infections, treatment of blood clots, and so forth.
Strength usually returns in a descending pattern, so that arm and
hand strength usually returns before leg strength. Often, right-handed
persons note more rapid return of strength to their left side and
vice versa. As arm strength returns, the patient is again able to
do things that used to be taken for granted, such as care for mouth
hygiene, cut meat and so forth. As ability to perform these routine
tasks returns, the successes can be emotionally quite fulfilling,
leading to outright crying with tears of joy. Rehabilitation in
many centres is accomplished by the coordinated efforts of several
groups of professionals. Their overall goal is to assist the patient
to return to as near normal a life style as possible. The specialist
in rehabilitation medicine usually coordinates and oversees the
total rehabilitation program.
An occupational therapist instructs the patient on exercises to
strengthen the upper limbs - shoulders, arms, hands and fingers.
They help to retrain or re-learn many activities usually taken for
granted such as holding a pencil, using a utensil, and so forth.
Muscle testing may be performed, and exercises designed to strengthen
the weaker muscles, such as the small muscles of the hands.
The physical therapist emphasises exercises to maintain tone and
strengthen lower limbs, and ultimately teaches the patient to walk
as independently as possible. A variety of methods are used to accomplish
these goals. Methods may include exercises in a pool where water
gives buoyancy; exercises on a mat with weights, stationary bicycle
pedalling and so forth. As leg strength improves various assistive
devices are used to provide balance and support during walking.
These may include parallel bars, walker, crutches and canes. Eventually,
if possible, independent walking without an assistive device is
accomplished. During this whole learning process emphasis is placed
on proper body mechanics, avoidance of substitution of stronger
muscles for weaker ones, and prevention of muscle strain.
In addition to occupational and physical therapists, other persons
may participate in rehabilitation. These may include nurses, as
well as social workers and psychologists.
The latter can play an important role to assist the patient and
family in dealing with the new and sometimes overwhelming problems
of paralysis, dependency, loss of income, and the associated multitude
of emotional problems. Emotional reactions to severe illness can
include frustration, depression, self-pity, denial, anger and so
forth. Since prognosis for the Guillain-Barré patient is relatively
optimistic, in spite of the potential gravity of the illness, a
practical approach is to take one day at a time during the rehabilitation
process.
There are a few differences between the Guillain-Barré and other
patients in the rehabilitation hospital that warrant comment. Most
of the patients in this kind of hospital have had a stroke, amputation
or brain or spinal injury and will regain some but limited return
of function. In contrast, the overall chance for return to a near
normal life style is rather good for the Guillain-Barré patient.
Furthermore, although most rehabilitation patients are strongly
encouraged to exercise to maximum tolerance, this is usually not
advised for the Guillain-Barré patient. Excessive exercise may lead
to aches, cramps, pain and exhaustion of muscles that have inadequate
nerve supply. Therefore, some moderation or pacing of exercises
is often advised.
As with most aspects of medical care, rehabilitation for Guillain-Barré
syndrome is individualised for the patient's particular problem.
PROGNOSIS
The overall outlook for the Guillain-Barré patient is relatively
optimistic. Although the exact percentages vary from study to study,
the following values give an estimate for long-term prognosis. Up
to 90% of patients reach nearly complete recovery. Some of these
patients may have persisting, but mild, abnormalities that will
not interfere with long term function. These may include abnormal
sensations, such as tingling, achy muscles or weakness of some muscles
that make walking or other activities awkward or difficult. Perhaps
5 to 15% of Guillain-Barré patients will have severe, long-term
disability that will prevent return to their prior life style or
occupation.
Rarely, a patient will be wheelchair bound for a prolonged time.
It is important to emphasise that, as in many aspects of medicine,
the prognosis or expectation for degree of recovery for any patient
cannot be predicted. Strength returns at various rates. Although
improvements may be noted from day to day, quite often they can
be appreciated on a week to week basis. As strength and endurance
increase, improvements may occur at a slower rate and only be noted
on a month to month basis. Recovery may continue for anywhere from
six months to two or more years.
LONG RANGE PLANS
As the patient progresses with their rehabilitation program,
there may be a role to plan for multiple long-range problems.
These problems include learning to drive and use convenient parking,
re-employment, learning to pace activities, sexual activities,
limitations of the wheelchair-bound patient and so forth. A social
worker may assist in handling many of these problems.
The majority of patients who were in a rehabilitation centre may
be placed on an outpatient therapy program when sufficient strength
has returned. At home, one level living may be temporarily helpful,
on a floor with a bathroom and a bed, until the patient is able
to climb stairs.
As sufficient strength returns, driver retraining may be appropriate,
especially if the patient has been hospitalised and not driving
for a long time. Driver retraining, and adaptation of an automobile
for hand controls, is available through some rehabilitation centres.
The frustration of physical exhaustion or shortness of breath associated
with prolonged walking may be reduced in the recovering patient
by parking near a building entrance, in a handicapped parking space.
A special parking placard or licence plate is available in some
states.
As the patient approaches the end of in-hospital rehabilitation,
it is usually appropriate to plan for re-employment. This is hopefully
a cooperative effort between patient, social worker, former employer
and, if available, a state bureau of vocational rehabilitation.
A potential barrier to return to work, as well as resumption of
a normal overall life style, is the onset, following a certain amount
of activity, of muscle aches, physical exhaustion, and abnormal
sensations, such as tingling and pain.
This problem may be circumvented by returning to work part-time
initially, and if possible, timing activity, such as walking, to
be intermittent with periods of rest on a couch or cot when exhaustion
or muscle aches occur or are anticipated. Many patients learn by
trial and error how much activity they can tolerate. For example,
as the author progressed through the day's activities he would experience
tingling of the right fourth and fifth fingers shortly before exhaustion
set in, thus signalling the time for reduced activity and rest.
After discharge from a formal hospital-based in- or outpatient
rehabilitation program there may still be a role or desire for continued
exercise. Usually, some of the physical and occupational therapy
exercises done as an in-patient can be performed at home. Also activities
of daily living, such as bathing, dressing, walking and stair climbing
may suffice as a practical outpatient exercise program. Should muscle
or joint cramps or aches develop after activity, over-the-counter
mild pain medications such as aspirin or acetaminophen (Tylenol®)
may provide relief. Since pain relief does not relieve the muscle,
tendon or joint strains, rest periods or a temporary reduction of
activity may be helpful.
Some caution is warranted with respect to non-hospital based exercise
programs, jogging and sports. Although these activities are popular,
their benefit and safety for the still-recovering Guillain-Barré
patient is questionable. Patients who engage in these activities
are capable of exerting beyond the physical limitations of their
tendons and muscles. Muscle tears as well as stress fracture of
bones can result and may require prolonged casting. Obviously these
injuries should be avoided by common sense pacing of activities
until the patient is recovered.
Upon return to home, the recovering Guillain-Barré patient can
usually resume their prior sexual activity. Positions that minimise
muscle exertion, such as lying on the back, may prevent exhaustion
until pelvic and other muscle strength has improved.
For the rare patient who is wheelchair bound, architectural barriers
may be overcome by using ramps to enter the home and other buildings.
One floor living may be required unless an elevator is available.
A visiting nurse and physical therapist may be utilised to treat
the patient at home. The significantly handicapped patient is referred
to their local rehabilitation centre.
The above review is only meant to provide guidelines. Each case
of Guillain-Barré syndrome is different. Each case requires individual
evaluation and treatment. This is usually accomplished under the
direction of participating doctors, including family physician,
internist, physiatrist and neurologist.
SUMMARY
Guillain-Barré syndrome, also called acute idiopathic polyneuritis
(rapid onset of inflammation of many nerves of unknown cause)
is a disorder that consists of weakness and even paralysis of
muscles of the legs, arms, and other parts of the body, as well
as abnormal sensations. It frequently follows a viral infection.
The illness can present in several ways, at times making the diagnosis
difficult to establish in its early stages. Early care is often
given in an intensive care unit so that potential complications
can be treated quickly should they occur.
No specific treatment is yet available to predictably stop the
illness' downhill course or reverse it. Corticosteroids have been
tried with varying results. Plasmapheresis, or removal of the liquid
portion of blood from the body, holds promise as a method to hasten
recovery. In the early stages of the illness treatments are directed
at preventing complications of paralysis. If breathing muscles become
paralysed, a comprehensive rehabilitation program in an appropriate
centre is often utilised.
As muscle strength returns, efforts are directed towards returning
the patient to their former life style. Once recovered, one would
not expect a subsequent attack at any later date.
Patient care involves coordinated efforts of a neurologist, general
physician, GP, physiotherapist, occupational therapist, social worker,
nurse and psychologist or psychiatrist. Emotional support from family
and friends, and information about this rare disorder may help the
patient learn to deal with this frustrating, disabling and potentially
catastrophic illness. A particularly frustrating consequence of
this disorder is long-term recurrences of fatigue and/or exhaustion
as well as abnormal sensations or muscle aches. These problems can
occur following the exertion of normal walking or working and can
be alleviated or prevented by reduction of activity and rest.
Pertinent facts about Guillain-Barré syndrome include the following
(the figures are approximate):
- Frequency is about 1 to 2 cases in 100,000 population each year
(0.001 - 0.002%); the disorder is rare.
- About 50% of cases follow a viral illness.
- Diagnosis can be difficult in the syndrome's early stages.
- The disorder is not contagious.
- Half the patients initially develop abnormal sensations; 25%
present initially with muscle weakness (often difficulty walking);
25% present initially with both abnormal sensations and weakness.
- Rehabilitation is the major form of treatment.
- Recovery may occur over 6 months to 2 years or longer. It can
occur sooner.
- In the early stages of the illness, prognosis or long-term outcome
is not predictable.
- Up to 90% of patients eventually have complete or almost complete
recovery.
- Five to 15% of patients will have significant long-term disability
or handicap.
- Perhaps 35 to 45% of patients have long lasting, but mild, abnormalities.
- The frequency of death is about 1 - 5% usually due to respiratory
or cardiovascular complications.
MILLER FISHER SYNDROME
In 1956, M. Fisher reported three patients with what is now
referred to as Fisher's or Miller Fisher Syndrome. It also has
the outlandishly long but medically descriptive name, acute disseminated
encephalomyeloradiculopathy. It is considered a variation of Guillain-Barré
syndrome and is rather rare.
Most cases have occurred in adult males shortly after an upper
respiratory infection. The prominent features are weakness of the
eye movements, often with double or blurred vision, a clumsy walk,
and loss of deep tendon reflexes, such as knee jerks.
Other common accompanying complaints include facial weakness or
sagging, abnormal sensations (numbness, tingling, etc.,) generalised
weakness and slurred speech. Spinal fluid protein is elevated.
As with usual forms of Guillain-Barré syndrome, the cause of Miller
Fisher syndrome is not known and treatment with corticosteroids
has sometimes been tried.
CHRONIC INFLAMMATORY DEMYELINATING POLYRADICULONEUROPATHY
CIDP, or chronic inflammatory demyelinating poly-radiculoneuropathy,
shares many features with Guillain-Barré syndrome, and in fact,
is sometimes referred to as chronic or relapsing (recurring) Guillain-Barré
syndrome. However it is very much less common. Other medically descriptive
names by which CIDP is known include chronic idiopathic polyneuritis
and chronic relapsing (dysimmune) polyneuropathy. It is usually
clear from soon after the onset that a patient has CIDP because
of its chronic, ie gradually progressive course evolving over months
or years rather than days or weeks as in Guillain-Barré syndrome.
Some patients with CIDP do have periods of worsening and then improvement
and individual relapses are sometimes rather confusingly like Guillain-Barré
syndrome.
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