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Effects Recovery
Guillain-Barr?(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. 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. 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. 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.
Residual
Effects
Following
Guillain-Barre'
________________________________________________________________________
Gareth J. Parry
Consultant Neurologist Auckland Hospital, Professor of Neurology
University of Minnesota
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Guillain-Barre syndrome is a disorder whose excellent prognosis is
invariably emphasized. Widely accepted figures suggest that 75%-85% of
patients make a complete recovery, however, many of my patients have
complained to me of minor but annoying persistent symptoms continuing for
years after the initial paralytic event. Although I have made no systematic
study of the proportion of patients with these residual complaints it is
certainly more than the 15%-25% that the figures in the literature would
suggest. The great majority of studies of GBS outcome are based on telephone
interviews or retrospective chart reviews and seemingly minor complaints may
have been missed or disregarded. Thus, patients are often asked if they have
returned to their previous work or other previous activities but they may
not have been asked whether they have more difficulty performing their
former activities. A note of caution was sounded in one small study from Dr.
J. McLeod and his colleagues in Australia who objectively evaluated a small
group of 18 recovered GBS patients and found that half of them had residual
neurological abnormalities. Even then the residual abnormalities were
considered to be significant in only four patients. A recent important paper
from Dr. I.S.J. Merkies and colleagues in Holland (Neurology 1999;
53:1648-1654) has established that residual effects from both GBS and CIDP
are much more common than has been generally reported and that seemingly
minor neurologic abnormalities may still result in annoying disabilities.
The study used a validated index of fatigue severity to assess residual
disability. It included 83 patients who had suffered from GBS an average of
five years previously. About 80% of these patients experienced fatigue that
was considered severe enough to interfere with their life despite the fact
that the majority had normal strength or only minor weakness. They noted
also that the fatigue did not seem to improve over time; the fatigue index
score was the same in patients in whom many years had elapsed as it was in
patients whose acute illness had occurred only 6-12 months previously. This
paper provides sound scientific support for the validity of the observations
of my patients who regularly complain of fatigue even when they have
returned to all or most of their former activities and who are working full
time at their former jobs. Although their strength may be normal when they
are examined in the doctor's office they are clearly unable to sustain the
same level of physical activity that they had performed prior to their GBS.
A second under-appreciated symptom that may persist for many years is pain.
Certainly, severe disabling pain is very rare. However, a number of my
patients complain of persistent discomfort in their feet. The discomfort may
take the form of annoying paresthesias (tingling) or there may be a vague
aching discomfort. The symptoms have the same characteristics as typical
neuropathic pain in that they tend to be worse in the evening or at night
and are particularly annoying following days during which the patients have
been up on their feet a lot. The discomfort is not particularly responsive
to analgesics but usually does respond to drugs such as gabapentin or
amitriptyline, drugs typically used in the treatment of neuropathic pain.
However, these medications have to be taken on a daily basis to be effective
and one problem with deciding whether to treat this residual symptom is that
the discomfort is usually rather mild. Thus, patients may be daily irritated
by their symptom but be reluctant to take a drug every day for a symptom
that significantly bothers them only once or twice a month. I have seen no
mention in the medical literature of this phenomenon. It is possible that I
see a highly selected group of patients in my practice who had initially
been more severely affected and that the prevalence of this annoying
residual symptom is much higher in my patients than in the usual population
of recovered GBS patients. I would be most interested to learn whether the
group of patients reported by Merkies and colleagues also suffered from
minor persistent discomfort.
- The basis for both of these seemingly minor residual symptoms (fatigue
and pain) is probably axonal degeneration. During the acute illness the
predominant underlying pathology in most patients is segmental
demyelination, a completely reversible phenomenon. However, some degree
of axonal degeneration is almost invariable. As recovery occurs function
is restored by a number of mechanisms. Axonal regeneration of motor
axons probably plays very little role in restoration of function except
in the more severe cases. Rather, surviving axons send out small
branches called collateral sprouts that restore the nerve supply to
those muscle fibers whose nerves have been damaged. This process of
collateral sprouting is very effective at restoring strength to a muscle
but the efficiency of the muscle suffers - the muscle must work harder
to achieve its goals. Thus, fatigue may result even when there appears
to be full restoration of strength. On the sensory side, even a small
number of damaged sensory axons may be sufficient to generate
spontaneous discharges that are registered as pain or discomfort.
- It is entirely appropriate that the good outcome of GBS should be
emphasized during the acute illness. During this time, the patient is
losing control of many motor functions, sometimes including life
preserving functions, and constant reassurance from the attending
physicians plays a vital role in the recovery process. However, it is
equally important to be aware that residual problems are experienced by
"recovered" GBS patients. Acknowledgement that such residual
problems exist will go a long way towards helping patients deal with the
frustration of their incomplete recovery.
- More research is needed to discover an effective treatment for the
residual fatigue. In addition, since these persistent symptoms are
probably related to the degree of axonal damage that occurs at the time
of the initial attack, we also need to continue to strive for earlier
and more effective treatment of the acute stage of the disease so that
these residual problems are minimized.
Disability
After
?Recovery? From GBS
____________________________________________
Kleopas A. Kleopa, M.D., Neuromuscular Fellow
Mark J. Brown, M.D., Professor
Department of Neurology, University of Pennsylvania
School of Medicine, Philadelphia, PA
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Guillain-Barre syndrome (GBS) is the most common cause of acute
neuromuscular paralysis in developed countries. Most patients recover and
return to productive, independent lives. In a recent representative survey
of 140 GBS patients, 70% made a complete neurological recovery within a
year, 22% could walk but were unable to run, 8% were unable to walk
unaided, and 2% remained bedridden or ventilator-dependent after a year.
Thus, despite the good prognosis for recovery, GBS can cause long-term
disability. Persisting disability is largely the result of weakness from
the motor nerve injury that occurred during the acute illness. An
estimated 25,000 to 50,000 persons in the United States alone are
experiencing residual effects from the disease. Most research on GBS has
focused on understanding the cause and finding better treatments. Much
less attention has been paid to the long-term disability caused by GBS. In
addition to the previously mentioned residual weakness, there may be pain,
fatigue, psychosocial dysfunction, possible relapses of the illness, and
late progression of weakness.
- Pain
- Moderate to severe pain is a well-recognized symptom during the
course of acute GBS. For some patients neuropathic pain, consisting of
abnormal painful sensations, may persist after recovery from the
disease. In a recent prospective study of 55 GBS patients followed for
up to 24 weeks, pain occurred during the course of the illness in
almost 90% of cases. Whereas deep aching back and leg pain were the
most common early on, abnormal painful sensations and myalgic-rheumatic
type pain were observed during the recovery period. Musculoskeletal
pain was common in association with physiotherapy. Painful abnormal
sensations in the extremities tended to persist after 8 weeks, and
were still present in some patients after 24 weeks. In two cases the
pain was severe. Overall, pain can be effectively relieved with an
escalating regimen of analgesic medications, starting with
nonsteroidal anti-inflammatory drugs or acetaminophen, and if
necessary including oral or parenteral opioids. Even severe pain can
be controlled, sometimes with the addition of patient-controlled
analgesia. In a large series of GBS patients treated for pain, these
medications were generally effective, and no adverse effects on
breathing function or narcotic addiction occurred.
- Chronic fatigue
- Fatigue following GBS is underrecognized by neurologists and
rehabilitation physicians, because attention is directed toward the
more objective weakness and sensory disturbances. In a recent study of
83 patients recovering from GBS, severe fatigue was reported as one of
the three most disabling symptoms by over 80%. The incidence of
fatigue did not correlate with age, or motor and sensory residual
deficits, but fatigue was more common in women. Fatigue was unrelated
to the time since the acute phase of the GBS, a median of 5.2 years in
this group. Another study of 123 GBS patients, evaluated 3 to 6 years
after the acute illness, concluded that psychosocial functioning,
especially in areas such as sleep and rest, alertness, emotional
behavior and social interaction, was seriously affected. This was true
even when "complete" physical recovery was reached, or only
minimal residual deficits were present. Deconditioning and less
engagement in physical activities were discussed as possible
explanations for persistent fatigue. A supervised training program and
low-intensity aerobic exercise may reduce daily fatigue, with
improvements in activities of daily living and functional capacity.
Specific treatments for other factors associated with fatigue, such as
sleep disturbances, pain, and daytime inactivity, are available.
- Psychosocial dysfunction
- Reports of long-term psychological sequela after GBS are rare,
although this issue may be a major factor in psychosocial dysfunction
of patients recovering from the disease. Many psychological factors
could contribute to chronic fatigue and social dysfunction, including
fear of disability, inability to cope with physical limitations, and
depression following a major illness. The role of depression in
psychosocial dysfunction after GBS is not fully understood. The
sickness impact profile of GBS survivors was found to differ from the
profile of other patients with depression. Nevertheless, further study
of the long-term psychological impact of the disease is necessary, and
depression should be considered on an individual basis when
appropriate. Both supportive psychotherapy and/or pharmacologic
treatment can be effective.
- Post-traumatic stress disorder (PTSD) has been reported in a patient
following severe GBS with paralysis and a prolonged intensive care
stay. The GBS-induced PTSD shared the features of PTSD seen following
other traumatic events. Even such profound psychological problems
following GBS can be treated with supportive psychotherapy and
appropriate medications. They may at least in part be prevented by
adequate pain management and the use of a communication system, such
as clear lucid letter-board in the event of near complete paralysis.
Better understanding, prevention and treatment of these issues may
have a positive impact on the quality of life for GBS survivors.
Moreover, it is important for patients and their families to know that
their psychosocial problems are also experienced by other patients
after GBS.
- Recurrence of GBS
- Although GBS is thought to be a one-time disease, relapses and
chronic recurrent forms can occur. Patients are often concerned about
the risk of having additional episodes of GBS. In a study of 220 GBS
patients, 15 were found to have a relapsing course, with one to 4
recurrent episodes. The interval between episodes ranged from 3 months
to 25 years. Antecedent events such as a viral infection preceded most
relapses, and patients presented each lime with the typical clinical
and laboratory findings of acute GBS. All patients had long
asymptomatic periods between the episodes. In a more recent study of
476 patients following GBS, 2.5% experienced a recurrence of the acute
illness, with a mean period of 16 months between the episodes (range
2-47 months). One patient had three episodes. The authors found no
relationship between the risk of having a recurrent episode and the
severity of the first episode. Furthermore, the severity of the
subsequent episode did not correlate with the intensity of the first
episode. Reaching a correct diagnosis may be challenging in these
cases. Even GBS experts may find it difficult to separate a
"relapsing variant of GBS" from chronic inflammatory
demyelinating polyneuropathy (CIDP), especially early in the course.
Recurrent episodes of true GBS, although rare, may occur following
similar preceding illnesses, and should be treated in the same way as
the initial episode. They respond well to the same established
treatment modalities.
- Delayed progression
- Weakness from GBS reaches its maximum during the first two or three
weeks of the disease. This is the active or acute phase of the
illness. After a plateau period of days or weeks, recovery begins,
lasting between weeks and two years. During this time strength
improves steadily. Strength and sensory function plateau after about
two years. However, many decades after GBS, recovered muscles once
weakened by the disease may again grow weak. This is a slow process
that occurs over years, and may at first escape the patient's notice.
It is likely that this delayed weakness is the effect of the normal
gradual age-related nerve cell loss on muscles that have a reduced
reserve nerve supply from earlier GBS. The same phenomenon has been
observed after poliomyelitis ("post-polio syndrome") and
other forms of acute nerve injury. The incidence of slowly progressive
late weakness in GBS is unknown, but it is rare. When it does occur,
the patient's physician must recognize that the new weakness of
seemingly recovered muscles does not necessarily indicate a second
attack of GBS.
- For many patients recovering from GBS, residual motor or sensory
deficits may be only one aspect of the long consequences of the
disease. Other issues described here may have a considerable impact on
their quality of life. Effective treatments are available for most of
these problems.
- References available on request.
- Contact Dr. Kleopa at: kleopa@mail.med upenn.edu
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