CHAPTER 1
ABSTRACTGuillain-Barr syndrome is a heterogeneous condition with
several variant forms that is the most common cause of acute
flaccid paralysis in both children and adults. It is estimated that
the condition affects about 1 to 3 individuals per 100,000 persons.
Patients typically complain of increasing pain and weakness in the
limbs associated with tingling dysesthesias in the extremities. The
majority of patients with Guillain-Barr have been diagnosed with an
infection in the 3 weeks prior to the development of symptoms.
Other events that have been linked to the disorder include
vaccinations, surgery, and certain drugs. Therapy includes
immunotherapy to accelerate the recovery process as well as
specific therapies for individual patients such as pain relief.
INTRODUCTIONGuillain-Barr syndrome (GBS) is a heterogeneous
condition associated with immunemediated, reactive, self-limiting
peripheral neuropathies.It represents at least five different
entities that are associated with an increased concentration of
cerebrospinal fluid protein but a normal cell count and manifest as
systemic motor paralysis.Three of these forms predominantly affect
the motor system: acute motor axonal neuropathy (AMAN), acute
motor-sensory axonal neuropathy (AMSAN), and acute inflammatory
demyelinating polyradiculoneuropathy (AIDP).The other two variants
are Miller Fisher syndrome and acute pandysautonomic neuropathy.The
disorder, triggered by a preceding bacterial or viral infection,
causes respiratory failure requiring mechanical ventilation in
approximately 25% of cases.Mortality rates in patients requiring
mechanical ventilation are as high as 20%, with persistent
disability and persistent fatigue in 20% and 67% of patients,
respectively.
CHAPTER 2
DEFINITIONGuillain-Barr syndrome (GBS) is disorder in which the
bodys immune system attacks part of the peripheral nervous system,
described best as a polyradiculoneuropathy. With the widespread
eradication of poliomyelitis, GBS is the most common cause of acute
and subacute flaccid paralysis in infants and children. The
disorder is characterized by progressive, symmetrical, usually
ascending weakness, and diminished or absent reflexes starting from
legs. Diffuse pain also is a common presenting feature in many
children with GBS, at times delaying accurate diagnosis. GBS was
thought previously to be an inflammatory disorder that affected
only the myelin sheath, resulting in diffuse demyelination.
However, it is now recognized that the process can also attack the
axon, leading to degeneration of the nerve itself.After the first
clinical manifestations of the disease, the symptoms can progress
over the course of hours, days, or weeks. Most people reach the
stage of greatest weakness within the first 2 weeks after symptoms
appear, and by the third week of the illness 90 percent of all
patients are at their weakest.
EPIDEMIOLOGYGBS occurs year round and in all age groups. Adults
are affected more commonly than children. Some studies suggest that
males are more likely to be affected than females. The overall
incidence has been estimated to range from 0.4 to 2.4 cases per
100,000 per year, with 3,500 new cases per year occurring in the
United States. The incidence in children is lower, with estimates
between 0.4 and 1.3 cases per 100,000 per year. GBS can occur at
any age but is rare in children under the age of 2 years.
ETIOLOGYGuillain- Barr syndrome is an autoimmune disease in
which the immune system destroys the cover of peripheral nerves (
myelin fibers ) by blocking the transmission of nerve to muscle .
If this happens , the muscles do not have a response to a command
from the central nervous and can lead to weakness , numbness and
paralysis . The brain also receives sensory signals that lead to a
state of not feeling well , chills , pain and other sensations
.Some state / disease which precedes and may related with the GBS :
infection vaccination surgery Systemic diseases :o malignancyo
systemic lupus erythematosuso thyroiditiso Addison's disease
Pregnancy or the puerperiumGBS often associated with acute
non-specific infections. Incidence GBS cases associated with this
infection approximately between 56 % - 80 % , 1 up to 4 weeks
before neurological symptoms arise such as respiratory tract
infections above or gastrointestinal infection.
InfectionDefiniteProbablePossible
ViralCMVEBVHIV Varicella-zoster Vaccinia/smallpoxInfluenza
Measles Mumps Rubella Hepatitis Coxsackie Echo
BacteriaCampylobacter Jejeni Mycoplasma PneumoniaTyphoidBorrelia
B ParatyphoidBrucellosis Chlamydia Legionella Listeria
PATHOPHYSIOLOGYBecause this disease involving motor axonal
degeneration without inflammation , other terms of this syndrome is
acute axonal neuropathy motors ( AMAN) . Paralysis of the limbs
showed signs of LMN , bulbar paralysis sometimes accompanies such
tetraparalisis , even can also occur only cranial sensory and motor
paralysis are found .In the demyelinating form , segmental
demyelination of peripheral nerves met with inflammatory cells.
Guillain- Barr syndrome with axonal degeneration can occur without
demyelination or inflammation .Mechanisms of disease occurs due to
excessive response of T cells from the abnormal previous infection
. CD4 + helper - inducer T cells are important mediators of the
disease . Various specific antigens may be involved in this
response , including Myelin P - 2 and ganglioside GM1.
CLINICAL MANIFESTATIONGBS manifests as a rapidly evolving
areflexic motor paralysis with or without disturbance. The usual
pattern is an ascending paralysis that may be first notice as
rubbery legs. Weakness typically evolves over hours to a few days
and is frequently accompanied by tingling dysesthesias in the
extremities. The legs are usually more affected than the arms, and
facial diparesis is present in 50 % of affected individuals. The
lower cranial nerves are also frequently involved, causing bulbar
weakness with difficulty handling secretions and maintaining an
airway. Pain in the neck, shoulder, back, or diffusely over the
spine is also common in early stages of GBS, occurring in 50 % of
patients. Deep tendon reflexes attenuate or disappear within the
first few days of onset. Cutaneous sensory deficits (e.g., loss of
pain and temperature sensation) are usually relatively mild, but
functions subserved by large sensory fibers, such as deep tendon
reflexes and proprioception, are more severely affected. Bladder
dysfunction may occur in severe cases but is usually transient.
Autonomic involvement is common and may occur even in patients
whose GBS is otherwise mild. The usual manifestations are loss of
vasomotor control with wide fluctuation in blood pressure, postural
hypotension, and cardiac
dysrhythmias.SubtypeFeaturesElectrodiagnosisPathology
Acute inflammatory demyelinating polyneuropathy (AIDP)Adults
affected more than children; 90% of cases ; recovery rapid;
anti-GM1 antibodies (
