Presentation on Guillian Barre syndrome

1. GBS
Dr Faiz Mukthar C
PG REGISTRAR - CMT

2. • The syndrome was named after Georges
Guillain (1876-1961) and Jean Alexandre
Barré (1880-1967), French neurologists. André
Strohl (1887-1977), a French physiologist,
worked together with the both neurologists
and is the third author in the description done
in 1916, and for this reason the syndrome is
also referred as Guillain-Barré-Strohl
syndrome.

3. Clinical Clarification
• Guillain-Barré syndrome is acute, progressive,
monophasic paralytic neuropathy resulting from
an autoimmune response affecting the peripheral
nerves and their spinal roots, typically in the wake
of immune stimulation by an infectious disease.
– Campylobacter jejuni is the causative pathogen in
25% to 50% of adult patients.
– Albuminocytologic dissociation.
• Consists of high levels of protein in the cerebrospinal fluid
without an increase in cell counts
• Is present in 50% of patients in the first week and 75% of
patients by the third week after symptom onset

10. Classification
Acute
inflammatory
demyelinating
polyneuropathy
Acute motor
axonal
neuropathy
Acute motor-
sensory axonal
neuropathy
Miller Fisher
syndrome

12. Acute inflammatory demyelinating
polyneuropathy (AIDP)
• Immune injury occurs specifically at the myelin
sheath and associated Schwann-cell
components, causing vesicular degeneration of
the Schwann cells.
• Facial diplegia with paresthesias is a localized
form of this subtype.
• Most common subtype in Europe and North
America, excluding Mexico (90% of cases) 3
• Second most common subtype in China, Japan,
Bangladesh, and Mexico (22%-46% of cases)

13. Acute motor axonal neuropathy (AMAN)
• Immune injury occurs primarily to the axolemma itself,
leading to nerve conduction failure.
• Pharyngeal-cervical-brachial weakness—marked by
acute weakness of the oropharyngeal, neck, and shoulder
muscles—is a localized form of this subtype.
• Most common subtype in China, Japan, Bangladesh, and
Mexico (30%-65% of cases).
– There was a significant increase in this variant of Guillain-
Barré syndrome during the summers of 1991 and 1992 in a
rural area of China.
– This form is sometimes referred to as Chinese paralytic
syndrome.
• Second most common subtype in Western countries (5%-
10% of cases).

14. Acute motor-sensory axonal neuropathy
(AMSAN)
• Rare subtype with more pronounced sensorial
symptoms
• Accounts for 5% to 10% of cases in the United
States and Europe.

15. Miller Fisher syndrome
• Characterized by clinical symptoms relating to
dysfunction of the third, fourth, and sixth
cranial nerves.
• Typically presents as ophthalmoplegia with
ataxia and areflexia and as associated distal
paresthesia without weakness.
• Accounts for 5% of cases in Western countries.

16. Clinical presentation (AIDP)
HISTORY and
EXAMINATION
PRODROMAL
SYMPTOMS
•INFECTION
•TIME PERIOD
LIMB
WEAKNESS
•PROGRESSION
•TIME PERIOD
RESPIRATORY
SYMPTOMS
•SEVERITY
SENSORY
PAIN
ANS

17. Clinical presentation (AIDP)
History
• Prodromal period
– fever (52% of patients)
• Due to infection is associated with the presence of bulbar palsy (affecting
cranial nerves IX, X, XI, and XII) causing dysphagia, dysphonia, and
dysarthria
– cough (48%)
– sore throat (39%)
– Rhinorrhea (30%)
• Patients with sore throat or cough often develop ophthalmoplegia and
headache with facial palsy
– Diarrhea (27%)
• Patients with diarrhea rarely show ophthalmoplegia or bulbar palsy
• Diarrhea and abdominal pain are closely linked to Campylobacter
jejuni cause
• About two-thirds of patients had symptoms of an infection in the 3 weeks
before onset of weakness.

20. Clinical presentation (AIDP)
• Characteristic symptom is progressive weakness
of more than 2 limbs, with progression lasting
fewer than 4 weeks
• Weakness is classically described as ascending
and symmetrical, typically manifesting first in the
following:
– Lower limbs as both proximal and distal weakness
(56%)
– Legs and arms simultaneously (32%)
– Upper limbs (12%)

21. Clinical presentation (AIDP)
• Typically acute progression of limb weakness,
beginning as early as 12 hours after onset and
proceeding to its maximum clinical deficit in 2 to
4 weeks.
– Sensory and cranial nerve involvement often occurs 1
to 2 weeks after infection or other immune
stimulation.
– Accompanying facial weakness is typically bilateral.
– Disease nadir occurs within 2 weeks after infection in
80% of patients, in fewer than 4 weeks in 97%, and in
fewer than 6 weeks in all patients.

22. Clinical presentation (AIDP)
• Respiratory muscle weakness tends to progress slowly
and to be roughly correlated with the degree of
extremity muscle weakness.
– Inability to take a deep breath, associated with respiratory
failure, has been reported in 20% to 30% of patients
• Infants present with irritability, decreased movement,
and hypotonia, and they may present with respiratory
insufficiency.
• Older children present with fatigue and weakness and
have trouble walking, climbing stairs, and rising from the
floor; at the disease nadir, approximately 60% of children
are not be able to walk and 24% are not able to move
their arms.

24. Clinical presentation (AIDP)
• Pain precedes weakness in one-third of cases.
– Two-thirds of all adult patients experience pain
during the acute phase of the disease, especially
back pain; for approximately 50% of these, the pain
is severe.
– Pain may manifest as dysesthesia or muscular,
radicular, or arthralgic pain; pain is worse with
movement
– Pain is the initial complaint in up to 50% of
children.

25. Clinical presentation (AIDP)
• Symptoms of autonomic dysfunction include
the following:
– Orthostasis.
– Syncope or near-syncope.
– Urinary retention.
• Guillain-Barré syndrome may be the first
symptom of HIV infection when triggered by
this condition.

26. Clinical presentation (AMSAN)
• Exceptions.
– Abrupt onset of motor weakness and acute flaccid
paralysis
– Neuropathy is purely motor, although 10% of
patients report distal paresthesias.
– Cranial nerve involvement occurs less frequently
than in demyelinating polyneuropathy.
– Autonomic dysfunction is not present or is mild

27. Clinical presentation (AMAN)
• Similar to acute inflammatory demyelinating
polyneuropathy with more sensory
involvement; it is difficult to differentiate the
2 without neurophysiologic studies
• Has a more severe clinical course than acute
inflammatory demyelinating polyneuropathy

28. Clinical presentation (MFS)
• Associated symptoms include blurred vision
and dysarthria.
• Patients typically present with cranial nerve
involvement resulting in facial, oculomotor, or
bulbar weakness, which may extend to the
limbs.
• Distal paresthesia is closely associated with
this variant.

29. Physical examination(AIDP)
• Bilateral symmetrical flaccid muscular weakness;
typically begins in distal legs and rapidly progresses
toward the oral and nasal region
– In children, weakness initially may be asymmetrical.
– There is occasional bilateral involvement of facial,
oropharyngeal, and oculomotor muscles
• Hyporeflexia or areflexia is a hallmark of GBS (acute
inflammatory demyelinating polyneuropathy)
– Decreased tendon reflexes are common.
– 10% of patients with acute motor axonal neuropathy have
normal or brisk reflexes during the course of illness.
– Children usually lose reflexes during the first week of illness.

30. Physical examination(AIDP)
• Sensory examination in adults reveals mild decrease in touch
sensation in most patients
– Approximately 40% of children have loss of sensation (usually
distal).
• Facial paresis occurs in more than 50% of patients, usually
bilateral.
• Signs of autonomic dysfunction (usually occurring in advanced
or severe disease) include:
– Extreme hypertension or hypotension; occurs in 20% of patients.
• Wide variation (greater than 85 mm Hg) in systolic blood pressure from
day to day.
– Arrhythmias ranging from bradycardia to tachycardia
• Bradycardia may be severe, possibly causing asystole .
– Abnormal pupillary response to light.

31. Physical examination(AMAN)
Exceptions:
• More rapid progression in acute motor axonal
neuropathy.
• Sensory examination is normal.
• Patient usually has preserved normal reflexes,
with hyperreflexia at the peak of the illness:
– 10% have normal or exaggerated reflexes
throughout the illness
– 5% have intact reflexes on presentation but
become areflexic as the disease progresses

32. Physical examination(AMSAN)
Exceptions:
• Presents as acute inflammatory demyelinating
polyneuropathy with symmetrical muscular
weakness (often a profound quadriparesis),
decrease in peripheral sensation, and
areflexia.
• Rapidly progresses, with maximal deficit
observed by day 7

33. Physical examination (MFS)
Exception.
• Characterized by the triad of
ophthalmoplegia, ataxia, and areflexia
– Corneal reflex may be compromised.

35. Causes and Risk Factors
• Typically preceded by infection or other immune
stimulation that induces an aberrant autoimmune
response, targeting peripheral nerves and their spinal
roots.
• Two-thirds of cases are preceded by symptoms of upper
respiratory tract infection or diarrhea.
• Autoimmunity does not arise in 99% of people exposed
to GBS–associated infections .
• HIV-associated GBS has been observed; this neuropathy
generally occurs early in HIV infection, even at
seroconversion.
• GBS has been reported shortly after vaccination with
Semple rabies vaccine and different strains of Influenza A
virus vaccine (rare).

36. Causative infections
• Campylobacter jejuni infection is identified in up to 50% of patients.
– Associated with axonal GBS (AMAN) and with Miller Fisher syndrome.
• Cytomegalovirus is identified in 10% of patients.
– Associated with demyelinating GBS (AIDP).
• Epstein-Barr virus, Influenza A virus, Mycoplasma
pneumoniae, Haemophilus influenzae, Zika virus, and Chikungunya virus
– Epstein-Barr virus is associated with AIDP.
• Molecular mimicry between microbial and nerve antigens is highly
significant, especially in the case of Campylobacter jejuni infection.
– Both cell-mediated and humoral mechanisms are implicated in pathogenesis.
• Immunologic studies suggest that more than one-third of patients
generate antibodies against nerve gangliosides.
– 95% of those with Miller Fisher syndrome carry these antibodies.

37. Risk factors and/or associations
• Age
– Incidence rises by 20% for every 10-year increase in age.
• Sex
– More prevalent in males; elevated relative risk of 1.78.
• Genetics
– Not been identified but suspected, especially genes that govern immune response
and nervous system effects.
• Other risk factors/associations
– Drugs - Heroin, suramin, streptokinase, and isotretinoin.
– TNF-α antagonist therapy.
– Autoimmune disorders such as SLE.
• Procedures and conditions
– Surgery
– Epidural anesthesia
– Bone marrow transplant
– Immunizations
– Hodgkin disease
– Sarcoidosis.

38. DIAGNOSIS
History and
physical
examination
LABORATORY
CSF ANALYSIS
IMAGING
Gadolinium –
MRI
Functional
Testing
Nerve
conduction
studies

40. Diagnostics
• Brighton criteria
– Bilateral and flaccid weakness of limbs (100% of patients)
– Decreased or absent deep tendon reflexes in weak limbs (91% of patients)
– Monophasic course and time between onset and nadir is 12 hours to 28
days (97% of patients)
– Cerebrospinal fluid cell count fewer than 50/μL (100% of patients)
– Cerebrospinal fluid protein concentration greater than reference range
(49% of patients initially, 88% at 3 weeks)
– Nerve conduction study results consistent with 1 of the subtypes (99% of
patients)
– Absence of alternative diagnosis for weakness.
• The more Brighton criteria identified, the greater the likelihood of
Guillain-Barré syndrome.
• Ranges from level 1 (highest level of diagnostic certainty, with all
criteria met) to level 4 (diagnosed as GBS, possibly due to equivocal
findings or insufficient data for further classification)

42. Diagnostics
Perform a lumbar puncture in all patients when suspected.
• Albuminocytologic dissociation (combination of a normal
cell count and raised protein level in the spinal fluid) is a
classic sign, found in approximately 90% of patients at
peak disease, with these caveats:
– Found in only half of patients on initial analysis
– Normal protein level (especially when measured in week 1
after disease onset) does not exclude the diagnosis; 10% of
patients have normal CSF protein analysis.
– 15% of patients with the disease do show a mild increase in
cerebrospinal fluid cell count (5-50 cells/μL) 1
• If HIV is also present, pleocytosis is not an aberration but
the norm.

43. Diagnostics
• Electrodiagnostic nerve studies support the
diagnosis and provide prognostic information.
– Perform in all patients when available
– Can confirm the presence, pattern, and severity of
neuropathy
– Features of acquired demyelination are characteristic of
acute inflammatory demyelinating polyneuropathy
• MRI scans of the spine may be as useful as
electrodiagnostic studies in supporting the
diagnosis, especially in pediatric patients.

44. IMAGING
Gadolinium-enhanced MRI of the spine.
• Post–gadolinium nerve root enhancement
supports the diagnosis of Guillain-Barré
syndrome
• On gadolinium-enhanced axial T1-weighted
images, patients have thickening of the
intrathecal spinal nerve roots and cauda
equina, with enhancement of both the
anterior and posterior spinal nerve roots.

45. FUNCTIONAL TESTING
Electrodiagnostic testing
• Evident at 2 weeks after the beginning of weakness;
nerve conduction studies can be normal early in the
course of illness
• Increase diagnostic yield by obtaining
measurements of at least
– 4 motor nerves
– 3 sensory nerves
– F waves (conduction in the proximal portion of nerves).
– H reflexes (muscle reaction after stimulation of sensory
fibers in the innervating nerve)

46. Electrodiagnostic testing
Allows diagnosis of GBS and differentiation of type
• Acute inflammatory demyelinating polyneuropathy
– Evidence of demyelination
• Prolonged distal motor latency
• Reduced velocity of nerve conduction
• Prolonged latency of F wave
• Conduction blocks
• Increased temporal dispersion
– H reflex is absent in 97% of patients within the first week of
symptom onset
– Sural sparing pattern—the finding of a normal sural
sensory nerve response in spite of abnormal upper
extremity sensory nerve results—is an electrodiagnostic
test hallmark of demyelinating GBS (AIDP)

47. • Acute motor axonal neuropathy
– Decreased motor nerve conduction amplitudes
• Acute motor and sensory axonal neuropathy
– Decreased motor and sensory nerve conduction
amplitudes
• Miller Fisher syndrome
– In 75% of cases, the only abnormality is absence
of H reflexes

48. CSF
Interpretation of results
• Cytoalbuminologic dissociation supports the diagnosis when
present.
• Combination of normal cell count and elevated protein level is
present in approximately 50% of patients during the first week
of illness, increasing to approximately 75% in the third week
and approximately 90% at peak disease.
• Protein level may be within reference range in the first week
of the illness but may then rise to several grams per deciliter.
• Cell count is typically within reference range (85%) but can be 5
to 50 cells/μL in 15% of patients.
• If HIV is also present, pleocytosis is not an aberration but the
norm.
– Cerebrospinal fluid WBC count in HIV-positive patients ranges from
0 to 17 cells/mm³ 11

49. DIFFERENTIALS
Acute-onset
chronic
inflammatory
demyelinating
neuropathy
LYME DISEASE
Acute hepatic
porphyrias,
including acute
intermittent
porphyria
Vasculitic
neuropathy
Hyperkalemic
periodic paralysis
Hypokalemic
periodic paralysis
Acute cervical
myelopathy due
to transverse
myelitis or cord
compression
Ocular
myasthenia gravis

50. Differentials
Acute-onset chronic inflammatory demyelinating neuropathy
• Symmetrical peripheral sensory neuropathy and weakness caused by nerve
fiber demyelination; autonomic dysfunction is not a feature of chronic
inflammatory demyelinating neuropathy
• In 15% of patients, there is acute onset of neuropathy; the plateau is reached
within 4 weeks, similar to GBS.
• Subsequent relapsing or progressive course of disease with cranial nerve
involvement occurs in 16% of patients; the course of GBS is monophasic.
• Diagnosis is usually made clinically with support provided by the following:
– Electrophysiological studies demonstrating impaired sensory and motor
conduction
• Motor nerve conduction velocities slower than 40 m/second in median and ulnar nerves, or
slower than 30 m/second in the peroneal nerve.
• Terminal latency greater than 7 milliseconds in the median or ulnar nerves, or 10 milliseconds
in the peroneal nerve.
• Conduction block and/or differential dispersion of the compound muscle action potential
– Sural nerve biopsy showing more than 15% teased demyelinated fibers.

51. Differentials
LYMES DISEASE
– Neuropathic pain, facial palsy, and other cranial
neuropathies may be present in advanced, untreated
disease
– Neuropathy is often more asymmetrical than that of
patients with Guillain-Barré syndrome
– Classic initial manifestation is erythema migrans, also
known as a bull’s-eye rash, growing to a diameter of 5
to 30 cm in 80% of patients 30
– Confirm diagnosis by serologic testing, such as a
sensitive enzyme immunoassay or immunofluorescent
assay test
• All positive or equivocal specimens: Use a Western blot to test
for both IgG and IgM antibodies to Borrelia bacterial antigen

52. Differentials
Acute hepatic porphyrias, including acute intermittent
porphyria.
• Porphyrias caused by deficiencies in the activities of heme
biosynthetic pathway enzymes
• Peripheral neuropathy with motor weakness may be
present in acute hepatic porphyrias, as well as cranial
nerve involvement .
• These porphyrias are inherited and not precipitated by
another illness; abdominal pain is typically present
– Proximal muscles are predominantly affected in 80% of
patients, with onset in the upper limbs in 50% 33
• Confirm diagnosis by noting elevated levels of
porphobilinogen in urine or plasma

53. Differentials
Vasculitic neuropathy
• Inflammation or occlusion of blood vessels that can lead to
ischemic peripheral neuropathy
– Peroneal and ulnar nerves are commonly involved
• Typically much more painful than GBS.
• Women are affected more often than men (nearly 60% versus
40%), and average age at diagnosis is approximately 60 years.
• Confirm diagnosis by histopathology of a sural nerve or
superficial peroneal nerve biopsy and concomitant biopsy of
the gastrocnemius or peroneus brevis muscle; both will reveal
vascular inflammatory lesions with features including:
– Transmural vessel wall infiltration; leukocytoclasis; destruction of
the endothelium, internal elastic lamina, or smooth muscle cells in
tunica media; perivascular and vascular hemorrhage; or occlusion
of vessel lumen

54. Differentials
Hyperkalemic periodic paralysis.
• Autosomal dominant muscle channelopathy with periodic focal
weakness, often involving thigh and calf muscles (rarely, facial or
respiratory muscles) in response to rising serum potassium levels
(either at least a 1.5 mmol/L increase or a concentration above 5
mmol/L [5 mEq/L])
• Attacks may be precipitated by potassium loading, anesthesia,
rest after exercise, hunger, change in activity level, cold,
menstruation, emotional stress, pregnancy, illness, or
glucocorticoids
• Onset begins before age 20 years
• Attacks can last longer than 1 week
• Diagnosed by history; serum potassium level may be normal or
elevated during an attack

55. Differentials
Hypokalemic periodic paralysis
• Acute flaccid paralysis when serum potassium drops below 3.5
mEq/L
– May be due to a single gene mutation affecting potassium, sodium, or
calcium channels, or secondary to hyperthyroidism (acute
thyrotoxicosis), renal or gastrointestinal loss, or diuretic or other
drug use
• Paralysis may resemble GBS.
– Ascending paralysis can develop, with eventual impairment of
respiratory function
• Early morning symptoms (eg, paralysis occurring in the morning)
and weakness following a meal or strenuous exercise occurs in
almost 50% of patients
• Diagnosis can be confirmed by serum measurement of
potassium and resolution of symptoms with correction of
hypokalemia

56. Differentials
Acute cervical myelopathy due to transverse myelitis or cord
compression
• Bilateral spinal cord dysfunction developing over approximately 4 weeks
with a well-defined upper sensory level and no prior illness defines
transverse myelitis or a developing compression of the spinal cord.
– Also may present as a rapidly progressive paresis or paraplegia, starting with
the legs and sometimes progressing to involve the upper extremities
– Initially, reflexes may be lessened or absent only to become hyperreflexic
after a mean duration of 4 to 6 weeks
• Differentiating features include the following:
– Urinary urgency or retention is a typical early finding of myelopathy, which
is less common in GBS.
– In GBS, dysesthetic pain, involvement of the upper extremities and cranial
nerves, and absent deep tendon reflexes are more likely
• Diagnosis of myelopathy can be confirmed by clinical presentation and
evidence of acute inflammation on spinal MRI or spinal cord
compressive lesion.

57. Differentials
Ocular myasthenia gravis
• Antibody-mediated autoimmune disorder of the neuromuscular
junction; ocular weakness presents in 85% of patients with myasthenia
gravis as fluctuating ptosis and/or diplopia.
• Ophthalmoplegia and autoimmune origin are characteristic of Miller
Fisher syndrome as well.
• Differentiating characteristics include the following:
– Most patients with Miller Fisher syndrome can identify an infection
preceding symptoms
– Ocular myasthenia gravis has a subacute or chronic onset with fluctuations
in muscle weakness; reflexes are present.
• Diagnosis is confirmed by the following: 38
– Abnormal EMG results of the repetitive nerve stimulation test, typically in
a proximal or facial muscle, are characteristic of ocular myasthenia gravis
– Serologic testing for acetylcholine receptor antibodies in ocular
myasthenia gravis

58. TREATMENT
Goals
Diminish
functional
impact of
disease
Manage pain
Prevent or
reduce the
impact of
complications
Support
recovery of
function

59. Disposition
Admission criteria
• All patients are admitted for supportive care.
• Criteria for ICU admission
– ICU admission and mechanical ventilation are recommended in
patients with at least 1 major criterion or 2 minor criteria:
• Major criteria
– Hypercapnia (PaCO₂ above 48 mm Hg)
– Hypoxemia (PaO₂ below 56 mm Hg while the patient is breathing
ambient air)
– Vital capacity less than 15 mL/kg of body weight
– Negative inspiratory force less than −30 cm H₂O
• Minor criteria
– Inefficient cough
– Impaired swallowing
– Atelectasis

60. TREATMENT OPTIONS
• The mainstay of treatment of Guillain-Barré
syndrome remains adequate supportive care,
respiratory support when required, and
immunotherapy .
• IV immunoglobulin and plasma exchange.
– No difference exists between the 2 treatments in regard
to improvement in disability grade at 4 weeks, mechanical
ventilation duration, residual disability, or mortality.
– Plasma exchange is effective when given within 2 weeks of
illness onset in patients who are unable to walk; it is most
effective when given within 7 days of weakness onset 41

61. Patients who
cannot walk
unaided.
5 plasma exchange
sessions over 2
weeks, with a total
exchange of 5
plasma volumes.
IV immunoglobulin
daily for 5 days .
Patients who are
able to walk (but
not run).
2 plasma exchanges
of 1.5 plasma
volumes.
Treat with IV
immunoglobulin if
can walk and has
following criteria
Mildly affected
Rapidly progressive
limb weakness
Respiratory
impairment
Severe swallowing
difficulties
Autonomic
dysfunction

62. • Consider giving a second course of IV immunoglobulin
to patients who deteriorate after initial stabilization or
improvement (benefit has not been demonstrated
consistently).
• Patients with Miller Fisher syndrome do not
require immunotherapy, presumably because
they have good natural recovery.
– Consider IV immunoglobulin for patients with severe Miller
Fisher syndrome who have swallowing and respiratory
difficulties.
• Overall, IV immunoglobulin has been adopted widely
instead of plasma exchange owing to its convenience,
wide availability, and minor adverse effects 1
• Disadvantage of IV immunoglobulin is extremely high
cost, which decreases the chance that it can be used by
low-income patients, in low-income areas, and by
patients who are underinsured

63. TREATMENT OPTIONS
• Corticosteroid therapy, previously used widely to treat Guillain-Barré
syndrome, has been shown to be ineffective both alone and in combination
with IV immunoglobulin .
– Corticosteroids actually may slow recovery from Guillain-Barré syndrome, but
they may have a place in treatment of severe neuropathic or radicular pain
• Mechanical ventilation is required for the 20% to 30% of patients who
develop respiratory failure; endotracheal intubation/tracheostomy may be
necessary
• Provide pain relief for acute pain that is both nociceptive and neuropathic
in origin; a combination of medications is typically used together because of
the mixed nature of the pain.
– Use opioids cautiously as they can suppress respiratory drive, worsen
autonomic gut dysmotility, and increase urinary retention, which are often
present in patients with Guillain-Barré syndrome.
– Start therapy for neuropathic pain early in the course of the disease owing to
the delayed effect of the medications.
• Indicated medications include gabapentin, pregabalin, carbamazepine, and amitriptyline
• Use corticosteroids, if necessary, to address neuropathic or radicular pain

64. TREATMENT OPTIONS
• Provide prophylactic therapy for deep vein thrombosis or pulmonary
embolism risk.
• Manage effects of dysautonomia
– Most complications related to dysautonomia occur in patients with
respiratory failure and advanced generalized weakness
– Treatments include the following:
• Nasogastric tube to allow feeding
• Bladder catheterization to relieve urinary retention
• Laxatives to relieve constipation
• Management of hyponatremia
– SIADH is predictive of a poor outcome
• Bradycardia leading to asystole may require a cardiac pacemaker.
• Begin physical therapy early during the course of illness and start
rehabilitation when improvement is observed .
• In patients with facial weakness, prevent corneal ulceration (eg, use
lubricant eye drops).

65. DRUG THERAPY
• IV immunoglobulin therapy
– Before IV immunoglobulin therapy, check IgA levels because
patients with IgA deficiency (due to IgA antibodies) are at higher
risk of anaphylaxis.
– Main component of immunoglobulin therapy is IgG, and the
usual dose is 2 g/kg divided over 2 to 5 days.
• If the increase in IgG is less than 10.92 g/L, consider a second session.
• Calculate change in IgG by subtracting the baseline level from the level at 2
weeks.
– Infuse IV immunoglobulin in patients with renal dysfunction at
50% of the normal rate.
• Immune Globulin (Human) Solution for injection; Children and
Adolescents: 1 g/kg/dose IV daily for 2 days. Alternatively, 400
mg/kg/dose IV daily for 5 days has been used.
• Immune Globulin (Human) Solution for injection; Adults: In one study,
patients received IVIG 400 mg/kg IV daily for 5 days.

66. DRUG THERAPY
• Anticoagulants
– Heparin
• Heparin Sodium (Porcine) Solution for injection; Adults: 5,000
units subcutaneously every 12 hours.
– Enoxaparin
• Enoxaparin Sodium (Porcine) Solution for injection; Adults: 40 mg
subcutaneous daily.
• Opioid analgesics
– Possible adverse effects: suppression of the respiratory
drive and worsening of autonomic gut dysmotility and
urinary retention; use these drugs with caution.
• Epidural morphine infusions (1-4 mg bolus injections every 8-24
hours) have been used successfully 2

67. Plasma exchange therapy
• Removal of large-molecular-weight substances, including harmful
antibodies, from plasma; typically exchange with albumin
(preferred over fresh frozen plasma)
– 5 plasma exchange sessions (each exchange comprising 2-3 L of plasma
according to body weight) over 2 weeks is the standard course for
patients who are unable to walk unaided.
– Mildly affected patients (ie, Guillain-Barré syndrome disability score of
1-2) who are still able to walk may benefit from 2 plasma exchanges of
1.5 plasma volumes.
– Plasma exchange and IV immunoglobulin therapy are considered to be
equally effective
• Indication
– Standard treatment for patients with Guillain-Barré syndrome who are
unable to walk unaided 42
– Also a treatment option for patients who are mildly affected and still
able to walk.

68. • Prior use of IV immunoglobulin infusion
therapy
• Hemodynamically unstable patients
• Pregnant patients
• Sepsis
• Hypocalcemia
Contraindications
• Hematomas at venipuncture/line insertion
site
• Fluid overload
• Allergic or anaphylactic reactions to plasma
• Vasovagal episodes with fainting
• Hypocalcemia
• Thrombocytopenia
Complications

69. DISABILITY
• The Guillain-Barré syndrome disability score is
widely used to provide an objective measure of the
severity of illness:
– 0: healthy state
– 1: minor symptoms, capable of running
– 2: able to walk 10 m or more without assistance but
unable to run
– 3: able to walk meters across an open space with
assistance
– 4: bedridden or chairbound
– 5: requiring assisted ventilation for at least part of the day
– 6: deceased

70. COMPLICATIONS
• During the acute phase of the disease, pneumonia, sepsis, pulmonary
embolism, or gastrointestinal bleeding develop in up to 60% of intubated
patients with Guillain-Barré in the ICU .
• Autonomic dysfunction manifests in two-thirds of patients as ileus,
cardiac arrhythmias possibly leading to asystole, atelectasis, and
respiratory failure .
• Increased risk for deep vein thrombosis due to immobility
• Severe fatigue has been reported in up to 80% of patients after the disease
course.
– Women and patients over 50 years of age most frequently experience fatigue
after illness.
• One-third of all patients still experience pain 1 year after onset.
• Among severely affected patients, 20% remain unable to walk 6 months
after symptom onset.
– 33% of patients infected with Campylobacter jejuni have prolonged severe
disability.
• Patients may suffer from anxiety and/or depression due to persistent pain
and disability.

71. PROGNOSIS
• 1 year after neuropathy onset, 65% of patients make a near-
complete recovery and regain the ability to perform manual work
– Of the 35% who do not recover completely, about 8% will die in the
acute stage, usually from cardiac arrhythmias or pulmonary emboli .
– 5% of patients with Guillain-Barré syndrome who live in developed
countries die from medical complications.
– Miller Fisher syndrome typically resolves without affecting the
respiratory muscles and has a benign course.
• 2 or more episodes have been reported in 7% of patients.
– Mean interval between recurrences in these patients was 7 years.
• Prognostic factors indicating a worse outcome include:
– Advanced age.
– Respiratory failure, associated with a reduction in vital capacity of
greater than 20%.
– Guillain-Barré syndrome following Campylobacter jejuni infection.
– CMAP amplitudes within 0% to 20% of the lower limit of normal.

72. ERASMUS GULLIAN BARRE SYNDROME
SCORE
• Apply after 2 weeks of illness to predict those who will not be able to walk independently
at 6 months (recovery is largely complete at 6 months)
• First requires deriving a Guillain-Barré syndrome disability score at 2 weeks after onset of
weakness
• Total score ranges from 1 to 7
• Age at onset
– 40 years and younger: 0 points
– 41 to 60 years: 0.5 points
– Older than 60 years: 1 point
• Diarrhea (within preceding 4 weeks)
– Absent: 0 points
– Present: 1 point
• Guillain-Barré syndrome disability score (at 2 weeks into the illness)
– 0 or 1: 1 point
– 2: 2 points
– 3: 3 points
– 4: 4 points
– 5: 5 points
– Scores of 3 points or less: 0.5% of these patients will be unable to walk without assistance at 6
months
– Score of 4 points: 7% of these patients will be unable to walk without assistance at 6 months
– Score of 5 points: 25% of these patients will be unable to walk without assistance at 6 months
– Score of 6 points: 55% of these patients will be unable to walk without assistance at 6 months
– Score of 7 points: 83% of these patients will be unable to walk without assistance at 6 months

73. Prevention
• Due to the association with Cambylobacter jejuni infection,
efforts at reducing the transmission of Cambylobacter
jejuni should reduce the incidence of Guillain-Barré syndrome,
particularly in less developed areas of the world. Reasonable
prevention measures include: 53
– Improving sanitation
– Cooking poultry products thoroughly
– Disinfecting water supply
– Avoiding raw milk consumption
• Patients who have previously developed Guillain-Barré syndrome
within 6 weeks of receipt of influenza vaccine may have influenza
immunization deferred, but it may be given if the provider feels
the benefit of protection (ie, reduction of influenza in patients
with high risk of complications from influenza) outweighs the
small theoretical risk of recurrence 1

76. Key Points
• Guillain-Barré syndrome is acute, progressive, monophasic
paralytic neuropathy resulting from aberrant autoimmunity, typically in
response to a causative infection
– Campylobacter jejuni is the most common precursory infection
• Diagnosis is made on the basis of clinical signs and symptoms, especially
the combination of hyporeflexia and symmetrical ascending weakness
peaking in 4 weeks or less.
– Miller Fisher syndrome, a rare subtype, is associated with ataxia and
ophthalmoplegia
• Albuminocytologic dissociation, consisting of high levels of protein in the
cerebrospinal fluid without an increase in cell counts, is characteristic
• EMG studies, which typically demonstrate demyelinating features,
support the diagnosis
• Treatment involves immediate hospitalization, immunotherapy with
plasma exchange or IV immunoglobulin, supportive care, close monitoring
for respiratory involvement (which may require mechanical ventilation),
and monitoring for dysautonomia (which may require hemodynamic
stabilization)

77. • During the progressive phase, autonomic dysfunction
manifests in two-thirds of patients, causing ileus,
arrhythmia, and/or contributing to respiratory failure.
– Pneumonia, sepsis, pulmonary embolism, and gastrointestinal
bleeding develop in 60% of intubated patients during acute
(progressive) disease 2
• Most patients (65%) have a good prognosis for recovery; in
developed countries, the fatality rate for patients suffering
from Guillain-Barré syndrome is 5% 3
• Long-term complications may include chronic fatigue
(80% 2), residual pain (33% 3), and inability to walk unaided
(20% of severely affected patients 4)

79. THANK YOU.....