Gullain barre syndrome

1. GUILLAIN–
BARRÉ
SYNDROME
Moderator: Dr Shivaprasada T
Student: Dr Srinivasa B J

2. CONTENT
• HISTORY AND INTRODUCTION
• ETIOLOGY
• EPIDEMIOLOGY
• PATHOPHYSIOLOGY
• EVALUATION
• TREATMENT / MANAGEMENT
• DIFFERENTIAL DIAGNOSIS
• PROGNOSIS
• REFERENCES

3. HISTORY AND INTRODUCTION-
• In the first beginning of first worldwar [28 July 1914 to 11 nov 1918] there
were only few neurologist in the war fields
• On 5th November 1914 in “the meetingof Sociétéde Neurologiede Paris’’ Augusta
Dejerine-Klumpke stated that ‘So many empty seats, dear colleagues, for
a first autumn session!’
• More than half of neurogist have joined the Army
• Georges Charles Guillain and Jean Alexandre Barré joined the “Centre
Neurologique” of the 6th army near AMIENS
• Immediately after two weeks, two young French soldiers were brought
with c/o paraesthesias, progressive fatigue, difficulty in walking

4. • These two cases were seen and examined by gullain and barre
and electrophysiological studies was done by André Strohl
• Thought Spinal roots and nerves, probably infectious or toxic
• Treated with massage and injections of strychnine
• Both patients recovered after a few months

5. • Two cases were presented in Medical Society of the
Hospitals in Paris on October 16th 1916 as
“Sur un syndrome de radiculonévrite avec
hyperalbuminose du liquide céphalorachidien sans
reaction cellulaire. Remarques sur le caractères cliniques
et graphiques des réflexes tendineux”
[syndrome of radicular neuritis with hyperalbuminosis of
the cerebrospinal fluid without cellular reaction]

8. • In 1850, Jean Baptiste Octave Landry de Théizillat
wrote “Note sur la paralysie ascendante aigue”
where he reported on 10 patients
• Landry did not perform a lumbar puncture, as
this was introduced by Quincke only in 1891.

9. • Sir William Osler in “The Principles and Practice of Medicine”
wrote about an ascending paralysis he referred to as an acute
infectious polyneuritis .
• In 1936 Alajouanine published a similar case in which
death had occurred

10. • Antonio Rodrigues de Mello reported a case of soldier without prodromal
symptoms, presented with a subacute ascending paralysis, slight sensory
disturbances in the limbs with paralysis of the trigeminal and facial nerves. The CSF
showed an ACD
• treated with intravenous and intrathecal vitamin B

11. DEFINITION
• Guillain-Barré syndrome (GBS) is an acute, frequently severe, and
fulminant polyradiculoneuropathy that is autoimmune in nature
characterised by
• Progressive symmetrical Proximal and distal weakness in legs
and arms
• Ascending type of weeknes
• Hyporeflexia/areflexia
• With/without sensory disturbances,
• Absence of CSF cellular reaction [Albumino cytological
dissociation]
• Usually preceded by Antecedent infection.

12. EPIDEMIOLOGY-
• Incidence world wide
• 10-20 cases per million annually,
(1-2 cases per 1 lac per year)
• WHO-incidence of GBS is 0.4 to 4.0 people per 100,000 annually.
• Slightly higher incidence in males,

13. • Affects all age groups
• Incidence ↑ by 20% every 10-yr increase in age after 1st decade.
• Adults > Children
• Regional variation-
• North America & Europe - demyelinating forms,
• Asia - axonal forms

14. PATHOGENESIS-
• Molecular mimicry and cross reactive immune response
• Target- Myelinated nerves
• Effect-
• Demyelination,
• Lymphocyte infiltration,
• Complement activation,
• Axonal degeneration.

16. DEMYELINATION-
Focal inflammation-myelin/schwann cell
Exudation of plasma proteins into CSF
Blockage of electric saltatory conduction
[slow conductionmuscle weakness]
demyelination
Antibodies deposition and Complement activation
T-cells  Macrophages
Secondary axonal
degeneration-
Slow recovery and more
residual disability [demyelination starts
at nerve roots]

18. AXONAL LOSS
IgG and Complement deposit at nodes of ranvier
Direct axolemma injury at nodes
Reversal
outcome
Paranodal myelin detachment,
Node lengthening,
Sodium channel disruption,
Altered ion & water homeostasis
Conduction
block
Axonal degeneration
Key point-
Minimal inflammatory
infiltrates

20. ANTECEDENT EVENTS- INFECTIONS & VACCINES
1. C. Jejuni infection-
• Most common(25% cases)
• 60-70% AMAN & AMSAN and 30% AIDP
2. H. influenza A & B-
• 2nd most common(17 & 16% cases, respectively),
• Influenza B: Pure motor forms and More severe

21. 3. Covid-19-
Symptoms-More rapid and severe than typical for GBS
Estimated incidence of GBS in-
• COVID-19-positive patients- 47.9/1 Lac/yr
• COVID-19-positive hospitalised patients- 236/1 Lac/yr.3
Reference - Guillain-Barré syndrome and COVID-19: an observational
multicentre study from two Italian hotspot regions, Filosto Et al, BMJ, 2021

22. 4. HIV-
• Non-immunocompromised>any stage
• After acute seroconversion
• Following immune reconstitution from HAART,
• Similar clinical course and prognosis (as those without HIV)
5. CMV infection
6. Vaccinations-
• Influenza vaccine- 1 additional case per million.
• H1N1(2009) vaccine- 1.6 cases per million vaccinated
• Recombinant zoster vaccine- 3 cases per 1 million vaccinations(@>65yrs
age)

23. • COVID-19 VACCINE-
Vaccine
Unadjusted
incidence
(GBS cases per 1 lac
person years)
Adjusted incidence
(GBS cases per 1 lac
person years)
Adenovirus vector Vaccine
(janssen)
32.4 6.03
mRNA vaccine
(Moderna)
1.3 0.56
Reference- JAMA- Incidence of GBS after Covid-19 vaccination in Vaccine Safety
Datalink

24. • OTHER TRIGGERS-
• HODGKIN’S LYMPHOMA
• SLE,
• SARCOIDOSIS,
• BONE MARROW TRANSPLANT,
• MEDICATIONS- TNF-ALPHA INHIBITORS, TACROLIMUS, SURAMIN
• SURGERY & TRAUMA

25. CLINICAL FEATURES-
• Weakness
• Ascending type of Progressive symmetrical muscle
weakness
• Usually starts in lower limb but it may starts in upper
limb and facial muscles
• Flaccid prox+distal arms+legs weaknes

26. • DEEP TENDON REFLEXES-
• Reduced or absent DTRs in arms & legs(90% cases)[most
common]
• Normal DTRs- h/o diarrhoea rather than respiratory
antecedent infection
• Increased DTRs-
• Axonal forms
• Bickerstaff Brain stem Encephalitis

27. • Cranial nerve/bulbar symptoms-
• Facial n. palsies-50% cases
• Oropharyngeal weakness-50% cases
• Occulomotor weakness-15% cases
Severe respiratory
weakness in up to 30%
cases

28. • Sensory Symptoms-
• Paresthesia ( ≈ 80% cases )
• During acute phase- Pain in back and extremities
( ≈ 2/3 cases )
• Dysautonomia-
• 38-70%
• Presentation-
• Ileus,
• HTN/hypotension,
• Tachy/bradycardia
• Urinary retention
Asso. with more frequent-
Cardiac arrhythmias,
Hyponatremia,
Higher burden of disability
6% mortality

29. UNCOMMON FEATURES-
• Papilloedema
• Facial myokymia
• Hearing loss
• Meningeal signs
• V. cord paralysis
• Post. Reversible leukoencephalopathy syndrome

30. TIME COURSE
Symptom onset
from few days-week from disease
onset
Recovery(months-yr)
Plateau/nadir by 4 wks(90% cases)
Disease progress over 2 wks(typically)(upto
4wks)
4-8wks-SIDP,
>8wks-CIDP
[<24hr or
>4wks=alternate
diagnosis]

32. VARIANTS OF GBS-
• Common-
1. AIDP(most common)
2. AMAN
3. AMSAN
4. MFS
5. BBE
6. PCB weakness
• Uncommon-
1. Paraparesis
2. Pure sensory form
3. Acute pandysautonomia
4. Acute bulbar palsy
5. Facial diplegia & distal
limb paresthesia

35. ACUTE MOTOR AXONAL
NEUROPATHY
• Antecedent C. Jejuni infection
• Young Asians
• Pathology- axonal injury
• DTR- preserved
• Sensory- spared

36. • Rapid progression
• Presentation- similar to AIDP
• Electrodiagnosis- reduction in CMAP amplitudes
• Antibodies- GM1, GD1a, GalNac-GD1a, GD1b

37. ACUTE MOTOR-SENSORY AXONAL
NEUROPATHY
• More severe form,
• Both sensory+motor fibres affected,
• Marked axonal degeneration
• Electrodiagnosis-
• Severely reduced/absent CMAP amplitudes
• Severely reduced/absent SNAP amplitudes
• Emg-
• Extensive active denervation
DTRs- preserved
Antibodies- same as AMAN

38. GQ1b Syndromes
• includes-
1. Miller fisher syndrome [MFS]
2. Bickerstaff Brainstem encephalitis [BBE]
3. Pharyngo-cervico-brachial weakness [PCB]

39. Miller Fisher Syndrome
• 5-10% in North America &
Europe
• 20% in Asia
• Incomplete forms-
• Ophthalmoplegia without
ataxia
• Acute ataxic neuropathy
without ophthalmoplegia.
MFS
ophthalmoplegia
ataxia areflexia
± fixed dilated pupils

41. • Anti-GQ1b antibodies-85-90%
• Electrodiagnosis-
• ↓/absent sensory response without slowing of sensory
conduction velocities
• If asso. with weakness(overlap syndrome)-
• Edx abnormalities of AIDP+

42. Bickerstaff Brainsten Encephalitis
• Facial weakness(45%)
• Bulbar symptoms(34%)
• Mild limb weakness(50%)
• Normal/brisk reflexes(40%)
• Edx-
• ↓ SNAP amplitudes
• Abnormal blink reflex
• No s/o demyelination
• Motor conduction
abnorm.(rare)
BBE
Encephalopathy ataxia
Ophthalmoplegia

43. Pharyngeal-Cervical-Brachial
Weakness
• Char. by acute weakness of
• Oropharyngeal muscles
• Neck muscles,
• Shoulder muscles,
• Swallowing dysfunction
• Auto-antibodies-
• GT1a(IgG)
• GM1 or GD1a
• Electrodiagnosis-
• Localized pattern of neuronal damage(similar to AMAN)

44. Variant Facial weakness
Legs strength &
reflexes
PCB weakness + Preserved
AIDP + Affected

46. Rare Variants-
• 1. Paraparesis-
• Mild type of GBS
• Char. by – weakness and hyporeflexia limited to
lower limbs at presentation
• Upper limbs -
• Intact strength in upper limbs
• Upper linb hyporeflexia
• Abnorm. NCS.

47. 2. Acute Pandysautonomia-
• Acute autonomic dysfunction + hyporeflexia
• Presenting features-
• Diarrhoea,
• Vomiting,
• Abdominal pain,
• Ileus,
• Orthostatic hypotension,
• Urinary retention,
• Invariant Heart Rate
• ↓ sweating/lacrimation/salivation
Reflexes- absent/reduced
May respond to immunotherapy

48. 3. Pure Sensory GBS-
 Isolated sensory abnormality(rare)
 Absent reflexes
 Minor Motor involvement,
 GD1b
 Subtypes(nerve pathology)-
• Acute sensory demyelinating polyneuropathy
• Acute sensory large fiber axonopathy-gangliopathy
• Acute sensory small fiber neuropathy-gangliopathy
D/D-
Paraneoplastic
sensory neuropathy

49. 4. Facial Diplegia & Distal limb Paresthesia-
 Acute onset B/L facial weakness + limb
paresthesia
 Other bulbar symptoms are absent
 Electrodiagnosis- Demyelinating pattern

50. 5. Acute Bulbar Palsy
 Ataxia
 Ophthalmoplegia
 Areflexia
 Facial weakness
 Neck and limb weakness-
absent
 Overlap with- MFS & PCB

51. Diagnostic Evaluation-
• NINDS GBS criteria-
• Required Features-
1) Progressive weakness
2) Areflexia/hyporeflexia(in weak limbs)
Supportive features-
1. Progression over days-4 weeks
2. Relatively symmetrical
3. Pain in trunk and limbs

52. Supportive Features-
4. Cranial nerve involvement
5. Autonomic dysfunctions
6. Mild sensory dysfunction
7. No fever at onset
8. CSF- typical
9. Electrodiagnosis - consistent with GBS
10.Recovery starting 2-4 weeks after progression
halts

53. Brighton Criteria-
1. Acute ,B/L, symmetrical limb weakness(flaccid)
2. ↓/Absent DTRs in weak limbs
3. Monophasic illness pattern
4. Absent alternate diagnosis for weakness
5. CSF- ACD
6. Electrodiagnosis- consistent with GBS
1-6
(all)
Level 1
1-4
+
5/6
Level 2
1-4
Level 3

54. [Roodbol et al] Levels 1, 2, and 3 had sensitivities
of 72%, 96%, and 98%, respectively

55. CSF Analysis-
P r o t e i n s -
• Usually 45-200mg/dl
• can be upto 1gm/dl
• CSF is often normal ≤48
• Varies by time since
symptom onset-
• 1st wk- present in 50-60%
• 3rd wk- present in >75%
C e l l s -
• Typically normal
• <5 cells/mm3
• May be upto 50 cells/mm3
• CSF pleocytosis-
• Concurrent HIV infection

56. Clinical suspicion of GBS
Acute onset progressive weakness
Hypo/areflexia
H/O antecedent infection(past 4
wks)
Evaluate for alternate diagnosis-
MRI brain & spine
NCS/EMG
Lab testing for other causes of acute
weakness
GBS unlikely
Lab screening to exclude other
common causes of acute
polyneuropathy
Do results suggest any alternate
diagnosis for acute polyneuropathy
LP for CSF analysis GBS unlikely
yes no
yes yes
no no
Continued..
Any atypical symptoms?
Do results suggest alternate diagnosis?

57. CSF- albuminocytologic
dissociation
(isolated ↑d proteins)
CSF >50 cells?
GBS unlikely-
Evaluate for alternate causes-
MRI brain & spine
Lab tests for acute weakness
Perform additional diagnostic
testing-
NCS/EMG
Repeat CSF(after 1 wk)
MRI spine with contrast
AntiGanglioside antibodies
Confirmed GBS
Confirmed GBS
yes
no
No(<50)
Yes(>50)
no yes

58. Electrodiagnostic studies-
• Not necessary if patient has typical presentation and ACD on CSF
analysis
• Serially
• Abnormal findings are most typically pronounced =3-4wk after
onset.
• AIDP-
1. Prolonged/absent F wave & Absent H reflex
2. Motor responses- ↑ distal latencies, conduction blocks,
temporal dispersion
3. Significant slowing/absent response on N. conduction velocities
4. Needle EMG- muscle weakness= ↓d recruitment/denervation

60. NCS Parameters Demyelinating Axonal
F wave latency Prolonged/absent
Absent
(but, no prolongation)
Distal motor latencies Increased Not significant
Conduction block Increased/+ ----
Temporal dispersion Increased/+ Not significant
Motor N. Conduction
velocities
Significant
slowing/absent
Not significant
Needle EMG
Reduced
recruitment/denervation
----
Distal motor+/-sensory
amplitudes
---- reduced

62. Additional testing-
• Indications-
1. Atypical symptoms
2. Equivocal results on CSF and Edx
• Includes-
1. Antibody testing
2. neuroimaging

63. Antiganglioside antibodies-
Subtypes & Variants IgG Antibodies to
GBS -
-AIDP/Facial Variant -(20-50% GM1-AIDP)
-AMAN & AMSAN GM1, GD1a
-PCB weakness GT1a>GQ1b>GD1a
MSF & Incomplete forms of MFS GQ1b, GT1a
BBE GQ1b, GT1a

64. Imaging-
• MRI brain & spine with contrast
• Bulbar symptoms ± Quadriparesis- MRI brain & Cervical
spine
• Lower limb weakness- MRI thoracic & lumbar spine
• MRI Findings-
• AIDP- Thickening and enhancement of intrathecal spinal N. roots &
Cauda (Involvement - ant. Roots > both ant. & Post. Roots)
• MFS-
• abnorm. Of post. Column,
• enhancement of CN3, CN6 & CN7.

65. Differential Diagnosis-
• Cerebral-
• Acute disseminated
encephalomyelitis
• B/L stroke
• Psychogenic symptoms
• Spinal-
• Transverse Myelitis
• Compressive neuropathy
• NMO
• Poliomyelitis
• Anterior Spinal Artery syndrome
• Peripheral Nerve lesions-
• Acute onset CIDP
• Critical illness polyneuropathy
• CMV asso. Radiculitis
• Diphtheria
• HIV related Radiculitis
• Lyme disease
• Metabolic
• Porphyria
• Thiamine Deficiency
• Tick paralysis
• Toxic Neuropathy & Vasculitis

66. • Cerebellar-
• Acute cerebellar ataxic
syndrome
• Post. Fossa structural
lesions
• NMJ disorders-
• Botulism
• Myasthenia Gravis
• LEMS
• NM blocking agents
• Muscle disease-
• Acute inflammatory
myopathies
• Acute viral myositis
• Acute rhabdomyolysis
• Critical illness myopathy
• Metabolic myopathies(↑/↓
K+)
• Mitochondrial myopathies
• Periodic paralysis
Differential Diagnosis-

67. Doubtful GBS?
• Weakness progress to nadir/plateau in <24hrs or >4wks
from onset,
• Sensory level,
• Asymmetrical weakness,
• B/B dysfunction @ onset, severe and persistent,
• Pulmonary dysfunction with little/no limb weakness @
onset,
• Isolated sensory signs(no weakness at onset),
• Fever @ onset,
• CSF leukocyte count >50 cells/mm3

68. Treatment of AIDP-
• Admission and monitoring-
1. Neurologic monitoring
2. Respiratory status monitoring,
3. Serial hemodynamic monitoring,

69. • Neurologic Monitoring-
• Muscle strength testing in
all 4 limbs,
• Facial, Neck & bulbar
strength assessment
(worseningMech.
Ventilator),
• Formal speech and
swallow assessment
• Respiratory status
monitoring-
• RR, O2 saturation, FVC,
Max. Inspiratory pressure,
Max, Expiratory pressure
• (worseningMech.
Ventilator)
• Hemodynamic monitoring-
• HR & rhythm, BP, fluid
status

70. Indication for ICU level Care-
• Impending respiratory failure-
•Rapid decline in resp. function(>30% in 24 hrs) with out
overt signs of resp. failure.
•Signs of Resp. Failure-
•Sob while speaking/@ rest,
•Accessory muscles use,
•Inability to count 15 in one breath,
•↑d RR with tachycardia or abnorm. ABG(Resp.
alkalosis),
•FVC-<15-20ml/kg or <1L
Elective Intubation

71. Severe Weakness-
• EGRIS Scale- predicts
the risk of respiratory
failure
• Score range- 0-7
• SCORE LEVELS-
• Low risk- 0-2
• Intermediate risk- 3-4
• High risk-5.7
Autonomic Disability-
• Labile BP,
• Arrhythmias.

72. EGRIS SCORE-
Measure Categories Score
Days between onset of weakness
and hospitalisation
>7 days 0
4-7 days 1
≤3 days 2
Facial &/or bulbar weakness at
hospital admission
Absent 0
present 1
MRC sum score at hospital
admission
60-51 0
50-41 1
40-31 2
30-21 3
≤20 4
EGRIS (total score) 0-7

73. Indications for Immuno-Modulatory
Therapy-
Symptom onset <4 weeks & unable to walk
independently with GBS Diability score-3-5
Symptom onset <4 weeks & rapid progression
Symptom onset <8 weeks & both severe and
progressing course

74. GBS Disability Score-
Features Score
Minor symptoms or signs of neuropathy, but capable of
manual work/running
1
Able to walk without support of stick, but incapable of manual
work/running
2
Able to walk with stick, appliance or support 3
Confined to bed/chair 4
Requiring assisted ventilation(for any part of the day/night) 5
Dead 6

75. Diagnosis of GBS
Indication for
Immunomodulatory
therapy?
Supportive care &
monitor.
Clinical detoriation with
indication for therapy?
Rehabilitation
IVIG or PLEX
Clinical Response?
Improvement
Inadequate response
Re-Evaluate
yes
no
no
yes

76. Re-evaluation
CIDp or other acute
polyneuropathy
Treat accordingly
Treatment Related
Fluctuation(TRF)
Retreatment with
Immunomodulator given
initially
[if <8 weeks]
Severe GBS
Supportive Care
[if IVIG given]
OR
PLEX retreatment
[if <4 weeks]

77. Supportive Management &
Monitoring-
• 30% cases develop neuromuscular Respiratory
Failure
• Autonomic Dysfunction require continuous
monitoring and medical intervention.
onset recovery
Progression plateau
2 weeks 2-4 weeks f/b

78. • 1. Neurological status-
• Serial neurological examination-
• at least daily during acute phase,
• Every 4-8 hrs if high risk of detoriation/ rapid worsening
• 2. Ventilatory Status-
• Monitoring Parameters-
• RR, O2 Saturation, FVC, Max. Inspiratory & Expiratory
Pressures.
• Frequency of Monitoring-
• O2 Saturation & FVC- every 2-4 hrs,
• (every 6-8 hrs if clinically stable for at least 2-3 days/ if mild
symptoms)

79. Indication for Intubation-
• RR >30bpm,
• SpO2 <92%,
• FVC <20ml/kg or <30% from previous value,
• MIP <-30cmH2O
• MEP <40cmH2O,
• Acute Hypercapnea with PaCO2 >50mmHg
• Others- Bulbar dysfunction with swallowing
difficulty, inability to clear secretions.

80. • 3. CVS & Autonomic Monitoring-
CVS-
• Labile BP  BP monitoring 4th hourly (till patient
improves)
• Brady/Tachy Arrhythmias  ECG
• [MC- sustained sinus tachycardia]
• Myocarditis
• Heart failure

81. Autonomic Dysfunction-
• Urinary retention,
• Ileus  Abdominal X ray, (ꝶ- neostigmine,
methylnaltrexone)
• Loss of sweating,
• Orthostatic hypotension,
• Paroxysmal Hypertension,
• Sustained Hypertension BP monitoring 4th hourly,

82. Management-
• Intra-arterial BP monitoring(if significantly fluctuating BP)
• Hypotension-
• IVF f/b Vasopressors
• Rule out- PTE, Hypoxemia, Sepsis, GI bleed, Fluid & Electrolyte
abnorm.
• Severe HTN(MAP>125mmHg)- Labet/Nicard/Nitroprusside,
• AV block & Asystole- Atropine/Cardiac Pacing
• 4. DVT Prophylaxis- pneumatic compression/compression
stocking,

83. • 5. Pain control-
• Neuropathic Pain- ꝶ- Gabapentin/CBZ,
• Somatic Pain- ꝶ- NSAIDs/Opioids,
• Severe/Refractory pain- ꝶ-Epidural Morphine,
• Pregnancy & GBS-
• Uncomplicated GBS does not affect pregnancy/labor.
• GBS does not increase in freq. during pregnancy,
• GBS- not an indication for C-Section
• Intra-Op- avoid S-choline,
• watch for exacerbation of neurodeficit & autonomic instability.

84. Immunomodulatory Therapy-
• Reduces time of onset of recovery by 40-50%,
• Indications-
Recommended for non ambulatory patients within 4
wks of symptom onset,
• Patient Selection-
1. Symptom severity-
• Unable to walk independently 10mtrs,
• Rapidly progressive weakness
• Respiratory/bulbar/autonomic involvement

85. 2. Duration since symptom onset-
• <4 weeks(ideal/better outcome)
• Severe GBS symptoms beyond 4 weeks(within 8 weeks)
3. Variant forms-
MFS
• symptoms evolve to include bulbar /limb weakness,
• Respiratory involvement
BBE
• immunoꝶ advised despite lack of evidence(d/t severity
of symptoms)

86. Selection of Agent-
• Available option(immunomodularoty)- IVIG & PLEX
• IVIG>PLEX
• Better tolerated, ease of administration, faster treatment
completion, less adverse effects.
Depends on-
• Local availability,
• Pt. preference,
• Risk factors,
• Contra-Indications

87. IVIG
• Dosing-
• 0.4g/kg/day for 5 days[total dose-2 gram/kg body wt.]
• Pre-treatment with 500ml NS+PCT
650mg+Diphenhydramine 25mg
↓
• Start infusion @ 0.3-0.5ml/kg/hr(to monitor adv.
Effects)
↓
• ↑ every 15-30min as tolerated upto 6-8ml/kg
• (any adverse effect↓ rate/stop infusion)

88. Adverse effects
• Hypotension,
• Transfusion reactions,
• Nausea, headache,
• Aseptic meningitis,
• AKI(sucrose containing products)
• Stroke/MI (hyper-viscosity)
• Anaphylaxis- IgA def.

89. CONTRA INDICATIONS
• Hyperviscosity
• Congestive heart failure
• Chronic renal failure (especially that due to
diabetes)
• Congenital IgA deficiency
Plasma exchange is preferred

90. Plasma Exchange(PLEX)
Administration-
• < 4 weeks of symptom-onset
• 4-6 treatments over 8-10 days, [Most effective - <1 wk of onset]
Overall efficacy, timing, & no. of exchanges-
• Superior to supportive care,
• Equally effective as IVIG,
• Most effective if started within 1 wk of onset,
• 6 exchanges≈4 exchanges>2exchanges

94. • Total Plasma Volume (TPV) to be removed
= total blood volume (TBV) ×(100% - Hct%)
• TBV is 5.5-7.5% of body mass and estimated as
70ml/kg for males and 65 ml/kg for female .
• Example:
Patient Wt:70 kg , Hct; 40% Patient
TBV: 70 kg x 70ml/kg = 4900ml
TPV: 4900 ml x (100% -40%) = 2940 ml
Plasma volume calculation

95. • Kaplan's equation
EPV= [0.065 x weight (kg)] x (1- Hct)
• A course of plasma exchange consist of 3-5 exchanges of 1-
1.5 volumes each, with an interval of 1-2 days between
procedures.

96. Replacement fluid
• 5% Albumin
• Most common used replacement fluid
• Dilute only with Saline
• GBS, MG, Goodpasture’s Syndrome
• Combination of saline and albumin
• FFP (Fresh Frozen Plasma)
• Used when necessary to replace clotting factors
• Typically used with TTP patients
• Cryo poor Plasma
• Cryoprecipitate has been removed

97. Others
GlucoCorticoids-
• not recommended
Eculizumab-
• Role- unclear,
• No recommendations for routine use.

98. Relapse/Worsening-
Immunotherapy
given
Symptoms fail to
improve/worsen
longer than 4wks
after onset
Re-Evaluate for
other acute
polyneuropathies
Initial improvement
f/b 20 detoriation
Treatment related
fluctuation(TRF)
[9-10% incidence]
OR
CIDP[2-5% incidence]

99. Role of Re-Treatment-
1. If symptoms fail to improve-
2. IF symptoms worsen after initial improvement-
• TRF <8 wks after onsetretreatment with same
immunomodulator therapy (effectiveness unclear)
Initial PLEX Re-treat with PLEX
No improvenent
Further detoriation
Initial IVIG Donot give IVIG
No improvenent
Further detoriation

100. Recovery & long Term
Management-
• Rehabilitation-
• Muscle strengthening exercises,
• Proper limb positioning & posture,
• Proper nutrition.
• Fatigue-
• Worse in Axonal forms,
• Persists for years
• Address contributors- depression, deconditioning,
• Graded supervised exercise program

101. Prognosis-
Long Term Outcome- by 1 year-
• ≈ 80% able to walk independently
• >50% have full recovery
• >10% have severe motor impairments,
• ≈5-10% have prolonged course with several months of
Ventilator dependency, very delayed & incomplete
recovery.
Mortality Risk-
• 3-7%(ventilated Pts- ≈20%)
• Highest during recovery,
• Resp./CVS complications

102. • Risk factors for Poor outcome-
• Age >60 years,
• Rapid onset of weakness,
• Severe muscle weakness on admission,
• Need for Ventilator support,
• Preceding diarrhoeal illness,
• Severe Edx abnorm.

103. REFERENCES
1. Harrison’s principles of internal medicine(21st Edition)
2. UpToDate
a. Guillain-Barré syndrome in adults: Pathogenesis, clinical features, and
diagnosis
b. Guillain-Barré syndrome in adults: Treatment and prognosis
3. Guillain-Barré syndrome and COVID-19: an observational
multicentre study from two Italian hotspot regions, Filosto Et al,
BMJ, 2021
4. Bradley’s Neurology in clinical practice , 7th edition
5. Adams and victor’s Principles of neurology , 8th edition

104. 4. Guillain-Barré syndrome, Van doorn et al., The Lancet, February 29,
2016
5. Guillain-Barre´ Syndrome, Peter Donofrio, American
Academy of Neurology, 2017
6. Guillain-Barre´ Syndrome, Shahrizalia et al., The Lancet,
February 26 2021
7. Tatu L, Bogousslavsky J (eds): War Neurology. Front Neurol
Neurosci. Basel, Karger, 2016, vol 38, pp 107–118 (DOI:
10.1159/000442597)
8. síndrome de Guillain-Barré, neurite