GBS

Complete neuron cell diagram

GuillainBarr syndrome (GBS), sometimes Landrys paralysis or GuillainBarr-
Strohl syndrome, is an acute polyneuropathy, a disorder affecting the peripheral
nervous system.Ascending paralysis, weakness beginning in the feet and hands and
migrating towards the trunk, is the most typical symptom, and some subtypes cause
change in sensation or pain as well asdysfunction of the autonomic nervous system. t
can cause life!threatening complications, in particular if the breathing muscles are
affected or if there is autonomic nervous systeminvolvement. "he disease is usually
triggered by an infection.
"he diagnosis is usually made by nerve conduction studies and with studies of
the cerebrospinal fluid. #ith prompt treatment by intravenous
immunoglobulins or plasmapheresis, together with supportive care, the ma$ority will
recover completely. Guillain%Barr& syndrome is rare, at '%( cases per ')),))) people
annually, but is the most common cause of acute non!trauma!related paralysis in the
world. "he syndrome is named after the *rench physicians Georges Guillain and +ean
Ale,andre Barr&, who described it in '-'..


In saltatory conduction, an action potential at one node of Ranvier causes
inwards currents that depolarize the membrane at the next node, provoking a
new action potential there; the action potential appears to hop from node to
node!


Classification
Si, different subtypes of Guillain%Barr& syndrome e,ist/
0
Acute inflammatory demyelinating polyneuropathy (A12) is the most
common form of GBS, and the term is often used synonymously with GBS. t is
caused by an auto!immune response directed against Schwann cell membranes.
Miller Fisher syndrome (3*S) is a rare variant of GBS. Accounting for
appro,imately 45 of GBS cases, it manifests as a descending paralysis,
proceeding in the reverse order of the more common form of GBS.. t usually
affects the eye muscles first and presents with the triad
of ophthalmoplegia, ata,ia, and arefle,ia. "he ata,ia predominantly affects the gait
and trunk, with the limbs relatively spared. Anti!G6'b antibodies are present in
-)5 of cases.
Acute motor aonal neuropathy (A3A7), also known as Chinese paralytic
syndrome, attacks motor nodes of 8anvier and is prevalent in 9hina and 3e,ico.
t is probably due to an auto!immune response directed against
the a,oplasm of peripheral nerves. "he disease may be seasonal and recovery can
be rapid. Anti!G1'a antibodies
:
are present. Anti!G1;antibodies are found more
fre<uently in A3A7.
Acute motor sensory aonal neuropathy (A3SA7) is similar to A3A7 but also
affects sensory nerves with severe a,onal damage. =ike A3A7, it is probably due
to an auto!immune response directed against the a,oplasm of peripheral nerves.
8ecovery is slow and often incomplete.
Acute panautonomic neuropathy is the most rare variant of GBS, sometimes
accompanied by encephalopathy. t is associated with a high mortality rate, owing
to cardiovascular involvement, and associated dysrhythmias. *re<uently occurring
symptoms include impaired sweating, lack of tear formation, photophobia, dryness
of nasal and oral mucosa, itching and peeling of skin, nausea, dysphagia, and
constipation unrelieved by la,atives or alternating with diarrhea. nitial nonspecific
symptoms of lethargy, fatigue, headache, and decreased initiative are followed by
autonomic symptoms including orthostatic lightheadedness, blurring of vision,
abdominal pain, diarrhea, dryness of eyes, and disturbed micturition. "he most
common symptoms at onset are related to orthostatic intolerance, as well as
gastrointestinal and sudomotor dysfunction (Suare> et al.
'--?). 2arasympathetic impairment (abdominal pain, vomiting, constipation, ileus,
urinary retention, dilated unreactive pupils, loss of accommodation) may also be
observed.
Bic!erstaffs "rainstem encephalitis (BB@) is a further variant of Guillain%
Barr& syndrome. t is characteri>ed by acute onset of ophthalmoplegia, ata,ia,
disturbance of consciousness, hyperrefle,ia or BabinskiAs sign. "he course of the
disease can be monophasic or remitting!relapsing. =arge, irregular hyperintense
lesions located mainly in the brainstem, especially in
the pons, midbrain and medulla are described in the literature. BB@ despite severe
initial presentation usually has a good prognosis. 3agnetic resonance
imaging (38) plays a critical role in the diagnosis of BB@. A considerable number
of BB@ patients have associated a,onal Guillain%Barr& syndrome, indicative that
the two disorders are closely related and form a continuous spectrum.
"hen an action potential arrives at the end of the pre#synaptic axon $yellow%,
it causes the release of neurotransmitter molecules that open ion channels in
the post#synaptic neuron $green%! &he combined excitatory and inhibitory
postsynaptic potentials of such inputs can begin a new action potential in the
post#synaptic neuron!


Signs and symptoms
"he disorder is characteri>ed by symmetrical weakness that usually affects the lower
limbs first, and rapidly progresses in an ascending fashion. 2atients generally notice
weakness in their legs, manifesting as Brubbery legsC or legs that tend to buckle, with or
without dysesthesias (numbness or tingling). As the weakness progresses upward,
usually over periods of hours to days, the arms and facial muscles also become affected.
*re<uently, the lower cranial nerves may be affected, leading
to bulbar weakness, oropharyngeal dysphagia (drooling, or difficulty swallowing andDor
maintaining an open airway) and respiratory difficulties. 3ost patients re<uire
hospitali>ation and about ;)5 re<uire ventilatory assistance for treatment of "ype
respiratory failure. *acial weakness is also common. @ye movement abnormalities are
not commonly seen in ascending GBS, but are a prominent feature in the 3iller!*isher
variant.
Sensory loss, if present, usually takes the form of loss of proprioception (position sense)
andarefle,ia (complete loss of deep tendon refle,es), an important feature of GBS. =oss
of pain and temperature sensation is usually mild. n fact, pain is a common symptom in
GBS, presenting as deep aching pain, usually in the weakened muscles, which patients
compare to the pain from over e,ercising. "hese pains are self!limited and may be
treated with standard analgesics. Bladderdysfunction may occur in severe cases but is
usually transient.
n severe cases of GBS, loss of autonomic function is common, manifesting as wide
fluctuations inblood pressure, orthostatic hypotension (a fall in blood pressure on
standing, leading to an increased risk of collapse), and cardiac arrhythmias.
Acute paralysis in Guillain%Barr& syndrome may be related to sodium channel blocking
factor in thecerebrospinal fluid (9S*). Significant issues involving intravenous salt and
water administration may occur unpredictably in this patient group, resulting in SA1E, a
cause of low sodium levels in the blood.

Cause
'edullated nerve fibers stained with silver nitrate!


All forms of Guillain%Barr& syndrome are due to an immune response to foreign antigens
(such as infectious agents) that is mistargeted at host nerve tissues instead, a
phenomenon calledmolecular mimicry. "he targets of such immune attack are thought to
be gangliosides, compounds naturally present in large <uantities in human peripheral
nerve tissues. "he most common antecedent infection is the bacterium Campylobacter
jejuni
:
followed by cytomegalovirus (93F).

Eowever, .)5 of cases do not have a known
cause. Some cases may be triggered by theinfluen>a virus, or by an immune reaction to
the influen>a virus. "here was increased incidence of Guillain!Barr& syndrome following
influen>a immuni>ation during the '-G.!'-GG swine flu pandemicH

however,
epidemiological studies since then have demonstrated either an e,tremely small
increased risk following immuni>ation (under ' additional case per million vaccinations)
or no increased risk.
"he end result of this autoimmune attack on the peripheral nerves is damage to
the myelin, the fatty insulating layer of the nerve, and a nerve conduction block, leading
to muscle paralysis that may be accompanied by sensory or autonomic disturbances.
n mild cases, nerve a,on (the long slender conducting portion of a nerve) function
remains intact and recovery can be rapid if remyelination occurs. n severe cases, a,onal
damage occurs, and recovery depends on the regeneration of this important tissue.
Appro,imately I)5 of patients have myelin lossH in the remaining ()5, the pathological
hallmark is a,on loss.
Guillain!Barr&, unlike disorders such as multiple sclerosis (3S) and =ou GehrigAs
disease (A=S), is a peripheral nerve disorder and does not in general cause nerve
damage to the brain or spinal cord.

(lectrical synapses between excitable cells allow ions to pass directly from one
cell to another, and are much faster than chemical synapses!


#iagnosis
"he diagnosis of GBS usually depends on findings such as rapid development of muscle
paralysis, arefle,ia, absence of fever, and a likely inciting event. 9erebrospinal fluid
analysis (through a lumbar spinal puncture) and electrodiagnostic tests of nerves and
muscles (such as nerve conduction studies) are common tests ordered in the diagnosis
of GBS.
9erebrospinal fluid
"ypical 9S* findings include albumino!cytological dissociation. As opposed to infectious
causes, this is an elevated protein level ('))%'))) mgDd=), without an accompanying
increased cell countpleocytosis. A sustained increased white blood cell count may
indicate an alternative diagnosis such as infection.
@lectrodiagnostics
@lectromyography (@3G) and nerve conduction study (79S) may show prolonged distal
latencies, conduction slowing, conduction block, and temporal dispersion of compound
action potential in demyelinating cases. n primary a,onal damage, the findings include
reduced amplitude of the action potentials without conduction slowing.
#iagnostic criteria
8e<uired/
2rogressive, relatively symmetrical weakness of two or more limbs due to
neuropathy
Arefle,ia
1isorder course J ? weeks
@,clusion of other causes (see below)
Supportive/
relatively symmetric weakness accompanied by numbness andDor tingling
mild sensory involvement
facial nerve or other cranial nerve involvement
absence of fever
typical 9S* findings obtained from lumbar puncture
electrophysiologic evidence of demyelination from electromyogram
$reatment
Supportive care is the cornerstone of successful management in the acute patient. Kf
greatest concern is respiratory failure due to paralysis of the diaphragm, the muscle
most important for breathing. ntubation may be needed when there is evidence of
impending failure of the muscles of breathing % when the vital capacity (F9) is less than
() mlDkg, the negative inspiratory force (7*) is less negative (i.e., closer to >ero) than
!(4 cmE(K, more than ;)5 decrease in either F9 or 7* within (? hours, rapid
progression of disorder, or autonomic instability.
Subse<uent treatment consists of attempting to reduce the bodyAs attack on the nervous
system, either by plasmapheresis, filtering antibodies out of the blood stream, or by
administeringintravenous immunoglobulins (Fg), to neutrali>e harmful antibodies and
inflammation causing disease. "hese two treatments are e<ually effective and a
combination of the two is not significantly better than either alone. Glucocorticoids have
not been found to be effective in GBS.

"reatment is usually begun as soon as the
diagnosis is made. 2lasmapheresis hastens recovery when used within ? weeks of the
onset of symptoms.

Fg has e<uivalent efficacy to plasmapheresis when started within
( weeks of the onset of symptoms, and has fewer complications.

Fg is usually used
first because of its ease of administration and safety profile. "he use of intravenous
immunoglobulins is not without risk, occasionally causing hepatitis, or in rare cases,
renal failure if used for longer than five days.
*ollowing the acute phase, treatment often consists of rehabilitation with the help of a
multidisciplinary team to focus on improving activities of daily living (A1=s).
Kccupational therapists may offer e<uipment (such as wheelchair and special cutlery) to
help the patient achieve A1= independence. 2hysiotherapists assist to correct functional
movement, avoiding harmful compensations that might have a negative effect in the
long run. "here is also some evidence supporting physiotherapy in helping patients with
Guillain%Barr& syndrome regain strength, endurance, and gait <uality,as well as helping
them prevent contractures, bedsores, and cardiopulmonary difficulties. Speech and
language therapists help regain speaking and swallowing ability, especially if the patient
was intubated or received a tracheostomy.
%rognosis
8ecovery usually starts after the fourth week from the onset of the disorder.
Appro,imately I)5 of patients have a complete recovery within a few months to a year,
although minor findings may persist, such as arefle,ia. About 4%')5 recover with
severe disability, with most of such cases involving severe pro,imal motor and sensory
a,onal damage with inability of a,onal regeneration. 1espite all improvements in
treatment and supportive care, the death rate is still about (%;5 even in the best
intensive care units. #orldwide, the death rate runs slightly higher (?5), mostly from a
lack of availability of life support e<uipment during the lengthy plateau lasting four to si,
weeks, and in some cases up to one year, when a ventilator is needed in the worst
cases. About 4%')5 of patients have one or more late relapses, in which case they are
then classified as havingchronic inflammatory demyelinating polyneuropathy (912).
2oor prognostic factors include age over ?) years, history of preceding diarrheal illness,
re<uiring ventilator support, high anti!G3' titre, and poor upper limb muscle strength.
&pidemiology
#orldwide, the annual incidence is about )..%? occurrences per ')),))) people. 3en
are one and a half times more likely to be affected than women. "he incidence increases
with ageH there are appro,imately ' cases per ')),))) people aged below ;) years and
about ? cases per ')),))) in those older than G4 years. "he incidence of GBS during
pregnancy is '.G cases per ')),))) of the population. 9ongenital and neonatal Guillain%
Barr& syndrome have also been reported.
'istory
"he *rench physician +ean =andry first described the disorder in 'I4-. n '-'., Georges
Guillain,+ean Ale,andre Barr&, and Andr& Strohl diagnosed two soldiers with the illness
and described the key diagnostic abnormality of increased spinal fluid protein production,
but normal cell count.
GBS is also known as acute idiopathic polyradiculoneuritis, acute idiopathic
polyneuritis, French polio, Landrys ascending paralysis and Landry Guillain Barr
syndrome.
9anadian neurologist 9. 3iller *isher described the variant that bears his name in '-4..