m Is an ë
 ëë

ë  

ë " IDP),
an autoimmune disorder affecting the peripheral nervous
system, usually triggered by an acute infectious process. The
syndrome was named after the French physicians Guillain, Barré
and Strohl, who were the first to describe it in 1916. It is
sometimes called Landry's paralysis, after the French physician
who first described a variant of it in 1859. It is included in the
wider group of peripheral neuropathies.

m It is frequently severe and usually exhibits as anascending

paralysis noted by weakness in the legs that spreads to the
upper limbs and the face along with complete loss of deep
tendon reflexes. With prompt treatment by plasmapheresis or
intravenous immunoglobulins and supportive care, the majority
of patients will regain full functional capacity. However, death
may occur if severe pulmonary complications and autonomic
nervous system problems are present. Guillain-Barré is one of
the leading causes of non-trauma-induced paralysis in the world.

m Guillain-Barré, unlike disorders such as multiple sclerosis "
S)

and Lou Gehrig's disease " LS), is a peripheral nerve disorder
and does not generally cause nerve damage to the brain or
spinal cord.
m Guillain-Barre syndrome is a rare
autoimmune disorder of the peripheral
nervous system. GBS is a
demyelinating disease, meaning that
segments of myelin are stripped from
their insulating position around
nerves, reducing the propagation of
electrical nerve impulses.This causes
loss of reflexes, muscle weakness,
and temporary paralysis "loss of
muscle strength).
 ëTIOLOGY
m Guillain-Barre syndrome is an autoimmune
disorder "the body's immune system attacks
itself).

m ëxactly what triggers Guillain-Barre syndrome

is unknown.

m It often follows a minor infection, usually a

lung infection or gastrointestinal infection.
Usually, signs of the original infection have
disappeared before the symptoms of Guillain-
Barre begin
 6
6

£ The annual incidence of Guillain-Barré is 0.4-
4.0 cases per population of 100,000

£ ëighty-five percent of patients recover with

minimal residual symptoms.

£ Death occurs in 3% to 8% of cases, resulting

from respiratory failure, autonomic
dysfunction, sepsis, or pulmonary emboli.

£ It is slightly more common in men than

women, and can affect people of any age, even
children
 SIX DIFFë ë
T SUBTYPëS OF GBS TH T ëXIST
m cute inflammatory demyelinating polyneuropathy " IDP) is the most
common form of GBS, and the term is often used synonymously with GBS. It
is caused by an auto-immune response directed against Schwann
cell membranes.

m
iller Fisher syndrome "
FS) is a rare variant of GBS and manifests as a

descending paralysis, proceeding in the reverse order of the more common
form of GBS. It usually affects the eye muscles first and presents with the
triad of ophthalmoplegia, ataxia, and areflexia. nti-GQ1b antibodies are
present in 90% of cases.

m cute motor axonal neuropathy "

), aka Chinese Paralytic

Syndrome, attacks motor nodes of anvier and is prevalent
inChina and
exico. It is likely due to an auto-immune response directed
against the axoplasm of peripheral nerves. The disease may be seasonal and
recovery can be rapid. nti-GD1a antibodies are present. nti-GD3 antibodies
are found more frequently in

.

m cute motor sensory axonal neuropathy "
S
) is similar to

but also

affects sensory nerves with severe axonal damage. Like

, it is likely due
to an auto-immune response directed against the axoplasm of peripheral
nerves. ecovery is slow and often incomplete.
m Acute panautonomic neuropathy is the most rare variant of GBS,
sometimes accompanied by encephalopathy. 6t is associated with a high
mortality rate, due to cardiovascular involvement, and
associated dysrhythmias. 6mpaired sweating, lack of tear
formation,photophobia, dryness of nasal and oral mucosa, itching and
peeling of skin, nausea, dysphagia, constipation unrelieved by laxatives or
alternating with diarrhea occur frequently in this patient group.
6nitial 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. The most common symptoms at onset are
related to orthostatic intolerance, as well as gastrointestinal and sudomotor
dysfunction (Suarez et al. 1994). Parasympathetic impairment (abdominal
pain, vomiting, obstipation, ileus, urinary retention, dilated unreactive pupils,
loss of accommodation) may also be observed.

m Bickerstaff¶s brainstem encephalitis (BB), is a further variant of Guillain±

Barré syndrome. 6t is characterized by acute onset of ophthalmoplegia,
ataxia, disturbance of consciousness, hyperreflexia or Babinski¶s
sign (Bickerstaff, 1957; Al-in et al.,1982). The course of the disease can
be monophasic or remitting-relapsing. Large, 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.Magnetic
resonance imaging (MR6) plays a critical role in the diagnosis of BB.

ëU O
S

TO
Y OF
ëU O

A nerve cell (neuron) consists of a large cell body
and nerve fibers²one elongated extension (axon)
for sending impulses and usually many branches
(dendrites) for receiving impulses. ach large axon
is surrounded by oligodendrocytes in the brain and
spinal cord and by Schwann cells in the peripheral
nervous system. The membranes of these cells
consist of a fat (lipoprotein) called myelin. The
membranes are wrapped tightly around the axon,
forming a multilayered sheath. This myelin sheath
resembles insulation, such as that around an
electrical wire. erve impulses travel much faster
in nerves with a myelin sheath than in those
without one. 6f the myelin sheath of a nerve is
damaged, nerve transmission slows or stops.

YëLI
SHë TH

TO
Y
D PHYSIOLOGY
The nervous system is divided
into the:

*Peripheral nervous system

(P
S)

*Central nervous system

(C
S)

The P
S consists of

‡sensory neurons running from

stimulus receptors that inform the

S of the stimuli
‡motor neurons running from the

S to the muscles and glands ±
called effectors - that take action.

The C
S consists of the

‡spinal cord and the

‡brain
I

U
ë SYSTë

P THOPHYSIOLOGY
 Predisposing factor Precipitating factor

‡Gender:
ale ¯Infection to

"
ale to female ratio is Campylobacter
1:5:1) jejuni
‡ ge
¯Poor hygiene
"Young adults age 15-35 ¯Stress
y-o) ¯Diet
"ëlderly age 50-75 y-o) ¯Life stye
 Infectious organism: invasion of Campylobacter jejuni via oral
route

To cause gastrointestinal infection "diarrhea & abdominal

cramping)

C. jejuni undergoes significant physiologic changes w/in the

intracellular environment to avoid mixture to lysosomal
enzymes w/c could eat & kill them

Immune system will

OLëCUL
I
IC Y response to the
intracellular invasion of
microorganism

DU L ëCOG
ITIO

Cell- Humoral
mediated immunity
immunity

istaken immune ctivates specific T
attack may arise Secrete
lymphocytes or T-cells
antibodies

Increased level of
Penetration of macrophage and ntibodies will
antibodies into basement membrane
lymphocytes level
fight foreign
around nerve fibers
microorganisms
T-cells released
lymphokines
Inflammation of the nerve cells

Lymphokines produced
Inflamed cells secrete cytotoxic macrophages activation
substances that affect or damage the
Schwann cells

Decreased myelin production

Ascending
paralysis
Dë
YëLI
TIO
Sensory
Impaired and
transmission 6mmobility of the
motor L
Tingling sensation of nerve loss
conduction
6nability to
umbness perform AL

Weakness of the

onstipation
L GU6LLA6 BARR
SY ROM
 CLI
IC L

IFëST TIO

Typical symptoms include

m Loss of reflexes in the arms and legs

m
uscle weakness or loss of muscle function "paralysis)
In mild cases, there may be no weakness or paralysis

ay begin in the arms and legs at the same time

ay get worse over 24 to 72 hours

ay occur in the nerves of the head only

ay start in the arms and move downward

ay start in the feet and legs and move up to the arms
and head
m
umbness, decreased sensation
m Sensation changes
m Tenderness or muscle pain "may be a cramp-like pain)
m Uncoordinated movement
 dditional symptoms may include

m Blurred vision
m Clumsiness and falling
m Difficulty moving face muscles
m
uscle contractions
m Palpitations "sensation of feeling heartbeat)

ëmergency symptoms "seek immediate medical help)

m Breathing temporarily stops

m Can't take a deep breath
m Difficulty breathing
m Difficulty swallowing
m Drooling
m Fainting
m Feeling light-headed when standing

U SI
G

Gë
ë
T
Supportive care with monitoring of all vital functions is
the cornerstone of successful management in the acute
patient. Of greatest concern is respiratory failure due to
paralysis of the diaphragm.

ëarly intubation should be considered in any patient with

a vital capacity "C) <20 ml/kg, a negative inspiratory
force "
IF) <-25 cmH2O, more than 30% decrease in
either C or
IF within 24 hours, rapid progression of
disorder, or autonomic instability.

Once the patient is stabilized, treatment of the

underlying condition should be initiated as soon as
possible. ëither high-dose
intravenousimmunoglobulins "IIg) at 400 mg/kg for 5
days or plasmapheresis can be administered, as they are
equally effective and a combination of the two is not
significantly better than either alone.
Therapy is no longer effective two weeks after the first
motor symptoms appear, so treatment should be instituted
as soon as possible. IIg is usually used first because of
its ease of administration and safety profile, with a total of
five daily infusions for a total dose of 2 g/kg body weight
"400 mg/kg each day). The use of intravenous
immunoglobulins is not without risk, occasionally causing
hepatitis, or in rare cases, renal failure if used for longer
than five days. Glucocorticoids have  been found to be
effective in GBS. If plasmapheresis is chosen, a dose of 40-
50 mL/kg plasma exchange "Pë) can be administered four
times over a week.

Following the acute phase, the patient may also need

rehabilitation to regain lost functions. This treatment will
focus on improving DL "activities of daily living) functions
such as brushing teeth, washing, and getting dressed.
Depending on the local structuring on health care, a team
of different therapists and nurses will be established
according to patient needs. n occupational therapist can
offer equipment "such as wheelchair and special cutlery) to
help the patient achieve DL independence.
 physiotherapist would plan a progressive training
program and guide the patient to correct, functional
movement, avoiding harmful compensations which might
have a negative effect in the long run.

speech and language therapist would be essential in the

patient regaining speaking and swallowing ability if they
were intubated and received a tracheostomy. The speech
and language therapist would also offer advice to the
medical team regarding the swallowing abilities of the
patient and would help the patient regain their
communication ability pre-dysarthria.

There would also be a doctor, nurse and other team

members involved, depending on the needs of the patient.
This team contribute their knowledge to guide the patient
towards his or her goals, and it is important that all goals
set by the separate team members are relevant for the
patient's own priorities. fter rehabilitation the patient
should be able to function in his or her own home and
attend necessary training as needed.

U SI
G DI G
OSëS
m Ineffective breathing pattern and
impaired gas exchange related to rapidly
progressive weakness and impending
respiratory failure
m Impaired physical mobility related to
paralysis
m Imbalanced nutrition, less than body
requirements, related to inability to
swallow
m Impaired verbal communication related
to cranial nerve dysfunction
m Fear and anxiety related to loss of
control and paralysis
 CO
PLIC TIO
S
£ espiratory weakness/failure "20-30% will need
intubation at some point during admission)
£ utonomic dysfunction in up to 65% including:
arrythmias, hypotension or hypertension,
fluctuating BP, , urinary retention.
£ Pain in up to 85%: typically back pain,and
musculoskeletal, the straight leg raise test will
be positive
£ Papilledema "secondary to high CSF protein)
£ DT
£ SI DH "26%)
£ cute enal Injury "secondary to IIG TX)
£ Hypercalcemia "secondary to immobility)
£ Hepatocelluar dysfunction
P TIë
TS WITH GBS

ëCOë I
G
F O
GBS