GBS - Guillian Barre Syndrome

Immune-mediated Polyneuropathy
L-GBS – Guillian Barre Syndrome
Dr. Dhananjay Gupta
Dept. Of Neurology
Ramaiah Medical College, Bangalore

Axonal/ Demyelinating
Axonal – Physical disruption of nerve
Toxic, Metabolic or Genetic causes

Axonal/ Demyelinating
Demyelination – loss or dysfunction of sheath
1. Entrapment/ compressive
2. Immunological - GBS
3. Toxic – diphtheria
4. Genetic – CMT

DYS-IMMUNE
POLYNEUROPATHY
ACUTE CHRONIC
AIDP
AMAN
AMSAN
Pandysautonomia
Mixed Motor Sensory Misc
- CIDP
- MADSAM
(lewis-sumner)
- MGUS
- WM
(anti-MAG)
MMN – CB CISP
DADS
CANOMAD
• Autoimmune
autonomic
neuropathy
• Vasculitic
neuropathy

1. AIDP
2. CIDP
3. MADSAM – lewis sumner
4. CISP

Definition
DEFINITION
 Prototypical Immune-mediated, monophasic,
 Poly-radiculo-neuropathy, characterised by
 Acute onset, areflexic, predominantly motor weakness, Progressing in
an ascending manner
*Lehmann HC, zu Horste GM, Kieseier BC, Hartung H-P. Pathogenesis and Treatment of Immune-Mediated Neuropathies.
Therapeutic Advances in Neurological Disorders. 2009;2(4):261-281. doi:10.1177/1756285609104792.
**Dimachkie MM, Barohn RJ. Guillain-Barré Syndrome and Variants. Neurologic clinics. 2013;31(2):491-510.

History
'The sensory and motor systems may be equally affected.
However the main problem is usually a motor disorder -a
gradual diminution of muscular strength with flaccid limbs
and without contractures, convulsions or reflex movements of
any kind. In almost all cases micturition and defaecation
remain normal. The first symptoms always affect the
extremities of the limbs and the lower limbs particularly.
When the whole body becomes affected the order of
progression is more or less constant: (1) toe and foot
muscles, then the hamstrings and glutei, and finally the
anterior and adductor muscles of the thigh; (2) finger and
hand, arm and then shoulder muscles; (3) trunk muscles; (4)
respiratory muscles, tongue, pharynx, oesophagus, etc.
Jean Baptiste Octave Landry de
Thézillat (1859)
Landry O. Notesur la paralysie ascendante gigue. Gazette Hebdomadaire. 1859;6:472–474.

Georges Guillain Jean-Alexander Barre Andre Strohl
1916 – French army doctors – 2 soldiers - hypo-reflexic weakness with raised
CSF protein levels, without a high WBC - ? Cause ?? Infection ?? Poisoning
History
*Guillain G, Barré JA, Strohl A. Radiculoneuritis syndrome with hyperalbuminosis of cerebrospinal fluid without cellular reaction. Notes on
clinical features and graphs of tendon reflexes- 1916. Ann Med Interne (Paris). 1999 Jan; 150(1):24-32.
** Guillain G, Barré JA. Paralysie ascendante gigue de Landry consecutive a Line vaccination antiryphoi-dique. Rev Neurol. 1919;3:595–598.

1949 – landmark description of 50 fatal cases.
Emphasized prominent damage to proximal nerves – at the junction of ventral and dorsal roots.
Unified the findings of Landry, Guillain, Barre and Strohl -- LGBS
History

1927 – Dragonescu and Claudian - GBS
Guillain – never believed the Landry ascending paralysis = GBS
: afebrile
: essential was raised CSF protein
: non- fatal, no respiratory distress in GBS
1958 – Charles Miller Fischer – acute external ophthalmoplegia
1965 – Thomas Munsat : “GBS is easy to diagnose but difficult to define
1969 – Asbury, Aranson, Adams – diagnostic criteria
History

Epidemiology
EPIDIMEOLOGY
 Incidence : 1-4/ 1,00,000 (*AAN)
 MCC of AFP in children
 MC acute NM-disorder seen in ICU
 M: F 1.1-1.7: 1
 Age : 2 months – 95 years
 Childhood GBS – average age 4-8 years

Epidemiology
 MC seen in 3rd – 5th decade
 Most cases in June-july, Sept-October *
 Seasonal variation – winters **
 Males > Females *
 Incidence higher in South-Asia as compared to America/ Europe
* Sharma G, Sood S, Sharma S. Seasonal, Age and Gender variation in GBS in a tertiary care centre in India. Neurosciences
and Medicine. 2013: 4, 23-28.
** Sudulagunta SR, Sodalagunta MB, Sepehrar M, et al. Guillain-Barré syndrome: clinical profile and management. German
Medical Science. 2015;13:Doc16. doi:10.3205/000220.
INDIA

* Kuwabara S, Yuki N. Axonal Guillain-Barré syndrome: concepts and controversies. Lancet Neurol 2013; 12: 1180–88
PREVALENCE OF AXONAL GBS

C. Jejuni GBS :
 pure motor axonal form (AMAN)
 More severe clinical disease,
 Less recovery chances
 More commonly a.w Anti-GM1 antibodies
Anti GM1 GBS
 rapidly progressive, severe neuropathy
 Predominantly distal distribution
 Motor
 No sensory/ cranial nerve disturbances

A Dutch study evaluated the relationship between vaccinations and GBS, more specifically recurrent
GBS. 245 GBS and 76 CIDP patients were studied with 106 GBS patients having received flu
vaccination (n=775). None of them had a recurrence of GBS after vaccination. Thus it was
concluded that flu vaccines are relatively safe. The most common symptoms post GBS and CIDP were
pain and fatigue.

Fehmi J, Scherer SS, Willison HJ, et al. Nodes, paranodes and neuropathies. J Neurol Neurosurg Psychiatry 17 August 2017

Node of Ranvier disruption as a cause of neurological diseases. Susuki K - ASN Neuro (2013)

* Kuwabara S, Yuki N. Axonal Guillain-Barré syndrome: concepts and controversies. Lancet Neurol 2013; 12: 1180–88

Presentation
Summary of Pathogenesis
1. AIDP :
Molecular mimicry  leading to lymphocytic mononuclear infiltrate of nerve roots,
peripheral nerves  with macrophage associated segmental demyelination
2. AMAN:
 paucity of lymphocytic infiltration
 sparing of the dorsal nerve roots, DRG, and peripheral sensory nerves.
 lengthening of the node of Ranvier  followed by the recruitment of macrophages
 Lengthening of node is reversible , results in acute conduction block neuropathy
 Late Wallerian degeneration

7-10 days prior to onset
of weakness
WEEKS ----

Nadir - 2 weeks – 50%
4 weeks – 90%
Recovery begins 2-3 weeks after
progression stops
WEEKS ----Ropper AH, Wijdicks EFM, Truax BT. In:Guillain-Barré Syndrome,Contemporary Neurology Series. Davis FA, editor. Vol.34. Philadelphia, PA: 1991.

The onset of the paralysis can be preceded by a general
feeling of weakness, pins and needles and even slight
cramps. The weakness spreads rapidly from the lower to the
upper parts of the body with a universal tendency to become
generalised. The paralysis then becomes generalised but more
severe in the distal parts of the extremities. When the
paralysis reaches its maximum intensity the danger of
asphyxia is always imminent. When the paralysis recedes it
demonstrates the reverse of the phenomenon which signaled
its development. The upper parts of the body, the last to be
affected, are the first to recover their mobility which then
returns from above downwards.'
Presentation
Jean Baptiste Octave Landry de
Thézillat (1859)
Landry O. Notesur la paralysie ascendante gigue. Gazette Hebdomadaire. 1859;6:472–474.

Presentation
1. Progressive ascending, areflexic* weakness
2. Pain/Paraesthesia often precede by 1-2 days
Lower back/ hip/ thigh pain is common – 50%
Finger tips should be paresthetic once sensory sx have ascended till midcalves
3. Weakness : starts in legs, proximal + distal muscles
Progress – UL --- neck, intercoastal, cranial muscles
Descending weakness/ onset in UL – 10-12%
4. Reflexes : 1st week – absent ankle --- progress to generalized
DTR invariable absent in limbs with power <3/5

Presentation
5. Cranial nerve palsies :
Facial weakness – 40-50% at presentation
Facial diplegia, maybe at onset or sequentially
 Paediatric : 9th 10th common
 Adults : 7th nerve most common
6. Autonomic dysfunction – 2/3rd at onset
MC – sinus tachycardia, less often bradycardia
facial flushing
fluctuating hypo/ hypertension
loss of sweating/ episodic diaphoresis
syncope/ pre-syncope/ retention/ constipation

Presentation
7. Oro-pharyngeal/ respiratory : 40%
1/3rd patients – may require mechanical ventilation
8. Bowel/ bladder/ sphincter disturbances
rare in early course of disease (<10%)
presence should raise a suspicion of myelopathy
9. MFS: Ophthalmoplegia, Ataxia, Areflexia
10. Psychotic – ONEIRIC hallucinations (bizzarre walking dreams)
After weeks of immobilization

AIDP AMAN
1. Infection CMV, EBV Campylobacter jejuni
2. Epidemics None China - children
3. Target Unknown GM1, GD1a
4. Onset – pain Common Absent
5. DTR Absent (preserved/
exaggerated in 5%)
Usually absent (preserved/
exagerrated in 20%)
6. Cranial nerve Common 60% Uncommon 20%
7. Sensory loss Frequent – 70% Absent usually
8.Autonomic involvement Frequent Rare
9. Recovery Relatively uniform 2 patterns – fast and slow
10. Electrophysiology Demyelination Axonal degeneration, CB

Variants
AMAN
China
Young population
Preceded by diarrhoea
Prognosis similar to AIDP
Mortality <5%
AMSAN
Abrupt onset
C. Jejuni diarrhoea
Respiratory distress common
Longer recovery
More residual deficits
Mortality 10-15%
MFS
Ataxia +Areflexia+
ophthalmoplegia
VARIANTS:
Acute MMN-CB
Reflexes unaltered
High titres anti- GM1

Variants
ANTI – GQ-1b
Ropper’s
variant, 1994

Presentation
Bickerstaff Brainstem Encephalitis (BBE)
1. Variant of MFS, Ataxia + Ophthalmoparesis +
2. Altered consciousness + paradoxical hyper-reflexia
3. Antecedent infection – 92%
4. Anti-GQ1b antibody – 66%
5. Elevated CSF protein – 59%
6. MRI abnormalities – 30%
7. Absent H-reflex may be the only finding in 75% MFS/BBE
* Yuki N, Sato S, Tsuji S, Hozumi I, Miyatake T. An immunologic abnormality common to Bickerstaff ’s brain stem encephalitis and Fisher’s
syndrome. J Neurol Sci. 1993;118(1):83–87.
** Ito M, Kuwabara S, Odaka M, et al. Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum: clinical
analysis of 581 cases. J Neurol. 2008 May;255(5):674–682.

Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barre syndrome. Ann Neurol
1990; 27 (suppl): S21–24. – NINDS criteria

Christiaan Fokke, Bianca van den Berg, Judith Drenthen, Christa Walgaard, Pieter Antoon van Doorn, Bart Casper Jacobs; Diagnosis
of Guillain-Barré syndrome and validation of Brighton criteria, Brain, Volume 137, Issue 1, 1 January 2014, Pages 33–43

Presentation
CONSIDER ALTERNATE DIAGNOSIS IF :
1. Fever at onset
2. Early, persistent bowel/ bladder disturbances
3. Marked asymmetry of weakness
4. A definite sensory level, significant sensory signs
5. Severe pulmonary dysfunction at onset
6. CSF pleocytosis >50 cells
7. Slowly progressive/ non-monophasic illness, without respiratory
involvement

Diagnosis
1. Lumbar Puncture: ACD
CSF is characteristically acellular (<10)
Or contain only few lymphocytes (10-50/mm) 80% patients
Proteins rise during 2-3 weeks (100-1000 mg)
IF CSF cells > 50 : consider
- HIV, Lymes, Sarcoidosis
- CMV polyradicupathy/ cauda equina syndrome
- Carcinomatous polyradiculitis, Aseptic meningitis

Peak in 4-6 weeks
Start to rise in 1-2 weeks
May persist at variable elevated
levels for many weeks
CSF PROTEIN LEVELS

Other CSF findings
Antibodies in CSF:
• IgG, IgM cerebroside – 38%
• IgG cardiolipin – 50%
• Gangliosides
GM1, GM2, GM3, GD1a, GD1b
• Heat shock proteins
HSP60, HSP70, HSP90
• Αβ- crystallin
Inflammatory mediators:
• Complement
C3a, C5a, C4b, C5-9
• Chemokines
IL17, IL18, IL22, IL37
• IL17, 37 correlate with disability
• Osteopontin levels – AMAN/ AIDP
• AIDP : IL1ra, IL8
• CIDP : CCL2, CCL7, CCL27, CXCL 9,10,12

Other CSF findings
Neuronal and Glial Markers:
• NfH – neuronal filament heavy chain
• NSE – neuron specific enolase
• S100
• 14-3-3 protein
• Glial fibrillary acetic protein, NfH,
S100 correlate with worse prognosis
Proteonomics:
↑ haptoglobin,
↓ transthyretin (usually neuroprotective
↑ cathepsin,
↓ cystatin C

Cerebrospinal fluid findings in Guillain–Barre syndrome and chronic inflammatory demyelinating polyneuropathies. ZSOLT
ILLES1, MORTEN BLAABJERG. Handbook of Clinical Neurology, Vol. 146 (3rd series)

Diagnosis
CSF findings in AMAN
 ACD may be +
 CSF/ SERUM albumin ratio – increased (~ AIDP)
 IgG index {CSF/SERUM IgG ÷ CSF/SERUM albumin} - increased (~ AIDP)
 CSF tau and NfH lvels correlate with poor outcomes

Diagnosis
CSF findings in MFS
 ACD may be +
 GQ1b – expressed in paranodal regions in of axons and in MNJ in CNS
 Presence suggests good response in CNS disease
 Most recover within 6 months
 CSF hypocretin-1 is reduced (similar to AIDP)
 CSF transthyretin and prostaglandin D2 synthetase not elevated

ROOT :
Prolonged or absent F-
response
Delayed or absent H-reflex
Axonal :Reduced CMAP
Prolonged distal latencies
Slowed conduction velocity
Distal conduction blocks

Diagnosis
2. Edx – NCS : “ Sural sparing “
 A normal sural sensory response in the setting of abnormal UL sensory
potentials, more commonly in the later stages
 Differentiates GBS from its mimics (infectious, toxic, metabolic and
spinal cord disorders.
 Specificity – 95%, Sensitivity – 41%, LLR positive – 8.20
* Derksen A, Ritter C, Athar P, et al. Sural sparing pattern discriminates Guillain-Barré
syndrome from its mimics. Muscle & nerve. 2014;50(5):780-784. doi:10.1002/mus.24226.

Diagnosis
3. MRI Spine (contrast)
 Surface thickening and Contrast Enhancement of
 Conus medullaris and cauda equina nerve roots
 Most common site of enhancement – ventral roots
 Though enhancement of posterior roots also seen
 MRI esp useful in paraparetic GBS to identify site of lesion
• Alkan O, Yildirim T, Tokmak N et-al. Spinal MRI findings of guillain-barré syndrome. J Radiol
Case Rep. 2009;3 (3): 25-8. doi:10.3941/jrcr.v3i3.153

Diagnosis
5. Metabolic abnormalities
 Raised CPK : in some
 ↑ ESR : infective/ neoplastic/ autoimmune cause
 LFT – abnormal in 10% : recent viral hepatitis – CMV/ EBV
 ↓ Na : SIADH – specially in ventilated patients, excess ANF
 ECG : Non specific ST-T changes may be seen
 Marked vomiting, hair loss, Mee’s line : Indicate Heavy metal toxicity

Differential Diagnosis
D/d:
1. AFP - Polio
2. Hypokalemic periodic paralysis
3. Thyrotoxic Periodic paralysis
4. N-M diseases – MG/ LEMS
5. OP poisoning
6. Botulism
7. Heavy metal toxicity (Lead)
8. Tick paralysis
9. MFS : MS/ VBI/ Encephalitis/ MG
Always Rule out Lymes disease/ HIV/ Syphillis/ metal toxicity

1. GBS
2. Lymes disease
3. CMV radiculitis
4. HIV radiculitis
5. Leptomeningeal
malignancy
D/D ACUTE ONSET LIMB WEAKNESS WITH/WITHOUT RESPIRATORY INVOLVEMENT
1. GBS
2. Toxic
3. Vascuilitic
4. Diphtheria/ Lymes
5. Porphyria
6. Thiamine deficiency
7. Metabolic/ electrolytes
8. HYPO-K/ Mg/ Po4/ glycem
NMJ disorders
1. MG
2. Botulism
3. Intoxications
Muscle disorders
1. Poly/ DM
2. Periodic paralysis
3. Criticle illness
myopathy
4. Rhabdomyolysis
5. Mitochondrial disease
Acute Spinal cord lesion

D/B GBS and TICK PARALYSIS – nearly impossible!!
1. Worldwide USA/ AUSTRALIA
2. Sensory loss may be + No sensory loss
3. CSF protein ↑ Normal
D/B GBS and PORPHYRIA
1. Mild pain Episodic Painful paralytic attacks
2. Predominant motor Sensory - motor
3. CSF protein ↑ Normal
D/B GBS and INFECTIVE (polio, West-Nile, enterovirus)
1. No fever at onset Episodic Painful paralytic attacks
2. More symmetrical Pure motor asymmetrical weakness
3. CSF acellular Pleocytosis
4. No meningitis Meningo-encephalitis syndrome +

D/B GBS and CARCINOMATOUS MENINGITIS with polyradiculopathy
1. Pain at onset + Painless
2. Acute Sub-acute
3. Mildly asymmetrical Fairly symmetrical
4. Proximal + distal Mainly distal, less proximal
5. Facial diplegia in 50% No facial weakness
6. Simultaneous weakness in limbs Sequential weakness in limbs
D/B cranial GBS/MFS and Myasthenia (bulbar)
1. Pain at onset + Painless
2. No diurnal variation Diurnal variation +
3. Mild sensory symptoms Pure motor weakness
4. Ocular muscles – advanced disease Early in disease
5. Mandibular muscles normal Exercised jaw hangs open
6. Areflexic DTR preserved

D/B cranial GBS/MFS and BASILAR ARTERY THROMBOSIS
1. Pain at onset +/- Painless
2. Areflexic Lively reflexes
3. Reactive pupils Sluggish/ locked in state
4. Plantar flexor Babinski +
5. F-wave abnormalities + Absent
D/B cranial GBS/MFS and Botulism
1. Preserved pupils Early Pupillary paralysis
2. Sinus tachycardia in 10% Bradycardia common
3. Ascending Descending paralysis

D/B GBS and CRITICAL ILLNESS NEUROPATHY
1. Acute Acute- subacute
2. Difficulty in weaning from ventilator
3. Sensory-motor Predominantly motor, mild sensory
4. Infections +/- Preceding sepsis/ overwhelming infection
5. Cranial nerve +/- Usually spared
6. Autonomic dysfunction +/- No dysautonomia
7. Demyelinating typically Primary axonal process
ICU patient with weakness and wiped out CMAPs, always consider toxic effects of
drugs, antibiotics and nutritional deficiencies in addition to the above two.
High dose steroids, NM-blocking agents can cause acute quadriplegic myopathy –
differentiated by high levels of CPK and myogenic potentials on EMG.

Treatment
TREATMENT
 GBS – a clinical diagnosis – symmetric
 Early intervention is essential to halt progression
 Immunotherapy is the standard of care
 In addition, supportive therapy

*Chevret S, Hughes RAC, Annane D. Plasma exchange for Guillain-Barré syndrome. Cochrane Database of Systematic Reviews 2017,
Issue 2. Art. No.: CD001798. DOI: 10.1002/14651858.CD001798.pub3.

Treatment
PlEx regimen :
 200 – 250 mL/Kg plasma removed
 In 4-6 treatment cycles, Usually on alternate days.
 Daily cycles can also be given, if no coagulopathy
 Replacement fluid : Saline + 5% albumin
 If FFP used – chances of infections, HIV, hepatitis
 S/E : hypotension, hypo-prothrombinemia, bleeding, arrythmias
* French Cooperative Group on Plasma Exchange in Guillain-Barré syndrome. Appropriate
number of plasma exchanges in Guillain-Barré syndrome. Ann Neurol. 1997;41:298–306.

1. No adequate trials comparing IVIG with placebo in adults (*moderate evidence)
2. In severe disease, IVIG started within 2 weeks, hastens recovery as much as PLEX
3. Adverse events are not significantly more with either treatment
4. But IVIG is significantly more likely to be completed
5. Giving IVIG after PLEX did not confer extra benefit (*moderate evidence)
6. More research is needed in mild patients and in whom treatment starts >2 weeks.

1. Data comparing IVIg with placebo in GBS is lacking ?well estb plasmaphresis
2. Lack of RCT for IVIg in children -- Expert Recommendation only!
3. IVIg has been found to be NON-INFERIOR to PLEX in trials
4. Dose of IvIg – 0.4 g/Kg/ day --- 3 vs 6 days --- no difference
5. No difference/ benefit of adding MePred to IVIg

Treatment
IVIG - MOA:
 Interferes with co-stimulatory molecules involved in APC
 Modulation of auto-antibodies, cytokines, adhesion molecules
 Modulation of the macrophage Fc receptor
 Disrupts complement activation and formation of MAC
 Upregulation of inhibitory Fc receptor – attenuates Ab-initiated inflammation
* Dalakas MC. Intravenous Ig in autoimmune neuromuscular diseases. JAMA. 2004 May 19;291(19): 2367–2375.

Treatment
IVIG - infusion:
 Total of 2g/Kg, Each bottle – 5gm
 Start slow infusion, @ 25-50 mL/ hour
 Watch closely for any reactions
 Every 15-30 minutes, Increase the rate slowly
 Increase the rate @50 mL/hour
 Subsequently, 1 bottle can be given over 4-5 hours

Treatment
Side effects of IVIG - rare:
 Headache
 Inflammatory local venous thrombosis at infusion site
 Anaphylaxis – esp. in IgA deficient patients
 Delayed red macular skin reactions of palms, soles, trunk, desquamation
 Renal failure (in dehydrated patients)
 Proteinuria
 Aseptic meningitis
 Hyperviscosity syndrome – stroke, MI, pulmonary embolism
(in old, immobile, DM, TCP, hypercholesterolemia patients)

Treatment
Severe Patients not improving: PlEx f/b IVIG
* The Dutch Guillain-Barré Study Group. Treatment of Guillain-Barré syndrome with high-dose immune globulins combined with
methylprednisolone: a pilot study. Ann Neurol. 1994 Jun;35(6):749–752.

1. Multi-centre double blind RCT --- 242 patients
2. IV MP 500 mg x 5 days vs placebo
3. Primary outcome – disability at 4 weeks
4. Steroids were shown to be INEFFECTIVE for treatment of GBS

*Hughes RAC, Brassington R, Gunn AA, van Doorn PA. Corticosteroids for Guillain-Barré syndrome. Cochrane Database of
Systematic Reviews 2016, Issue 10. Art. No.: CD001446. DOI: 10.1002/14651858.CD001446.pub5.
Moderate quality evidence show CS alone donot significantly hasten recovery from GBS or affect long-
term outcome. According to very low quality evidence, oral CS delay recovery. Diabetes requiring insulin
was more common and hypertension less common with corticosteroids based on high quality evidence.

* Wang, Y.-Z. et al. Action mechanism of corticosteroids to aggravate Guillain-Barré syndrome. Sci. Rep. 5, 13931;
doi: 10.1038/srep13931 (2015).

I-SID-GBS TRIAL – currently data being evaluated – results awaited
Large, prospective, open label, observation trial studying the effect of a second dose of IVIG
given shortly after the first dose.

JET-GBS TRIAL – Japanese Eculizumab Trial - GBS
prospective, multicenter, placebo-controlled, double-blind, randomized phase II
study conducted at 13 tertiary neurology centers, to evaluate
efficacy and safety of ECULIZUMAB (against C5) for GBS treatment.

Treatment
SUPPORTIVE THERAPY :
1. Monitor vitals, SBC, TLC
2. Tachycardia/ brow sweating
3. Pain – opiods/ gabapentin/ CBZ
4. High protein diet
5. Eye protection
6. ?paralytic ileus/ autonomic dysfunction
7. ?Bed sores
8. SIADH – 10-15%
9. DVT prophylaxis
10. Nosocomial infections

Treatment
Monitor Resp Insufficiency on Daily Rounds:
1. Severe impairment of resp. can occur before dyspnoea appears
2. Tachypnoea may indicate incipient resp. failure
3. Strength of neck muscles/ trapezii parallel Diaphragm weakness
4. SBC >20 : indicates Vital Capacity > 1.5 L.

Treatment
Managing Pain :
1. Most common residual abnormality – pain, cramps
2. Radicular back pain and neuropathic pain
3. NSAIDs, Opiods, Anti-depressants
4. Gabapentin, pregabalin, CBZ, tramadol, Mexilitene

Prognosis:
 Recovery starts – 4 weeks
 Avg period of mechanical vent – 22 days
 Time to walk unaided – 50 days
 Complete recovery – 200 days
 MC residual – pain, fatigue, cramps, depression
 20% patients cannot walk un-aided even after 6 months
 Axonal regeneration may take 6-18 months
 Most improvements happen during 1st year, minimal after that
Prognosis
* Drenthen J, Jacobs BC, Maathuis EM, van Doorn PA, Visser GH, Blok JH. Residual fatigue in Guillain-Barre
syndrome is related to axonal loss. Neurology 2013; 81: 1827–31.
* Merkies IS, Faber CG. Fatigue in immune-mediated neuropathies. Neuromuscul Disord 2012; 22 (suppl 3): S203–07.

Poor Prognostic factors:
x Age > 40
x Preceding diarrhoea (4 weeks), CMV, C. jejuni
x Rapid progression (<7 days)
x More duration of disease
x Higher CSF protein
x Higher anti-GM1 titres
x Severe reduced distal CMAPs (<20% LLN)
x Requirement of mechanical ventilation
x Erasmus GBS outcome score at 2 weeks ≥ 5
AMAN/ AMSAN : though less autonomic/ cranial, rapid muscle atrophy
Prognosis

Prognostic Scores – EGOS:
Prognosis
EGOS scores at 2 weeks and MRC sum
scores at 1 or 2 weeks have the
highest predictive value for
prognosis.
EGOS – simple score, based on 3
parameter, accurately predicts
outcomes
** van Koningsveld R, Steyerberg EW, Hughes RA, Swan AV, van Doorn PA, Jacobs BC. A clinical prognostic scoring
system for Guillain-Barre syndrome. Lancet Neurol 2007; 6: 589–94.

Prognostic Scores – mEGOS:
Prognosis
mEGOS scores – uses MRC sum
scores instead of disability scores
Can be used as early as 1 week
Age at onset, preceding diarrhoea
and MRC sum scores individually
a.w. unable to walk at 4 weeks, 3
months and 6 months
** Walgaard C, Lingsma HF, Ruts L, van Doorn PA, Steyerberg EW, Jacobs BC. Early recognition of poor prognosis in
Guillain-Barre syndrome. Neurology 2011; 76: 968–75.
Modified Erasmus GBS Outcome Score
Prognostic Factor Score Prognostic factor Score
Age at onset
<40
41-60
>60
0
1
2
Age at onset
<40
41-60
>60
0
1
2
Preceding diarrhoea
Absent
Present
0
1
Preceding diarrhoea
Absent
Present
0
1
MRC sumscores (admission)
51-60
41-50
31-40
0-30
0
2
4
6
MRC sumscores (day 7)
51-60
41-50
31-40
0-30
0
3
6
9
mEGOS SCORE 0-9 mEGOS SCORE 0-12

Prognostic Scores – EGRIS:
Prognosis
Erasmus GBS respiratory
insufficiency score – used to
predict respiratory depression.
Most of these score are tested in
AIDP and need to be validated for
children and axonal variants
** Walgaard C, Lingsma HF, Ruts L, et al. Prediction of respiratory insufficiency in Guillain-Barre syndrome. Ann
Neurol 2010; 67: 781–87.

Take Home Message:
► Besides the classical GBS, variants, forms fruste are known
► Treatment should not be delayed even if electrophysiology non-confirmatory
► All patients should be treated with IVIG/PlEx even In mild cases
► Therapy should be initiated within 2 weeks
► Though in worsening patients, treat even ≥ 2 weeks
► PlEx C/I in hemodynamically unstable patients
► IVIG – careful in patients with hypercoagulability or renal insufficiency
► No justification to use recurrent IVIG or PE unless recurrent disease
Prognosis

GBS - Guillian Barre Syndrome

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