RECOGNIZING and MANAGING

GUILLAIN-BARRÉ SYNDROME in
EMERGENCY SETTING

Baarid Luqman Hamidi

Department of Neurology
SEBELAS MARET UNIVERSITY SCHOOL OF MEDICINE
DR.MOEWARDI HOSPITAL
SOLO-INDONESIA

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PIN PERDOSSI, SOLO, 6-8 NOVEMBER 2014 2
 INTRODUCTION

• NEUROPATHY
MONONEUROPATHY
MULTIPLE (MONO) NEUROPATHY
POLYNEUROPATHY
POLY-RADICULO-NEUROPATHY

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 SYNONYMS : GULLAIN-BARRÉ SYNDROME

1 : Idiopathic Polyneuritis
2 : Acute Febrile Polyneuritis
3 : Infective Polyneuritis
4 : Post Infectious Polyneuritis
5 : Acute Inflammatory Demyelinating Polyradiculoneuropathy

6 : Gullain-Barré Strohl Syndrome

7 : Landry Ascending Paralysis
8 : Landry Gullain-Barré Syndrome 4
 HISTORY
• 1859 : Jean Baptise LANDRY, firstly wrote on GBS
Westphal introducing “Landry Ascending paralysis”
terminology
• 1916 : George Gullain Demyelinating Neuropathy 
Jean Alexandre Barré AIDP
Reported in changed of CSF :
Protein , cell normal
Andre Strohl
(DISSOCIATION ALBUMINOCITOLOGIQUE)
AIDP : Acute Inflammatory Demyelinating Polyneuropathy
• 1990 : Yuki et al (Japan)  discovered 2nd subtype of GBS
AMAN : Acute Motor Axonal Neuropathy
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 DEFINITION of GBS
GBS is a type of immunomediated
polyneuropathy affecting
peripheral nerves/radices, as an
important cause of acute
symmetrical weakness (of
spinal/cranial nerves)
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 DEFINITION G B S . . .
•Parry (1993) : GBS is a type of acute
ascending polyneuropahty, occuring 1-3
weeks after acute infection.
•Bosch (1998) : GBS is a clinical syndrome
characterized by acute flaccid paralysis
associated with autoimmune process on
targets peripheral nerves, radices and
cranial nerves.
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 EPIDEMIOLOGY

• GBS is a world wide disease

• Downing et al : frequency in
autumn
• Zhao Bao Xun : 60% GBS cases : July –
October (end of spring and autumn)

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 EPIDEMIOLOGY . . .

• Annual incidence 0,6-1,9 cases/100.000

• Mayo Clinic Medical Center (USA) : incidence
rate 1,7/100.000
• Age incidence : Peaks 15 – 35 years and
50 – 74 years
• Sex incidence : men ~ woman
• Race incidence : whites 83%, black 7%, Hispanic
5%, Asian 1%, others 4%
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 EPIDEMIOLOGY . . .

> 60

> 40 - 60

15 - 40

< 15 yrs

0 0.02 0.04 0.06 0.08 0.1 0.12 0.14 0.16

Figure 1. Age incidence (Annual coefficients of incidence)

Parry G.J. 1993

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 EPIDEMIOLOGY . . .

Europe
AIDP :
North America
GBS
subtype
East Asia
AMAN : Central America
South America

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 ETIOLOGY

Etiology of GBS has not been

definitely known

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• Diseases/conditions usually preceed/probably
have association with GBS :
1. Infection (50-80%) :
 URT infection 1 -4 weeks prior to GBS
 GI tract infection
2. Vaccination
3. Surgery
4. Systemic diseases:
 Malignancy
 Systemic Lupus Erythematosis
 Thyroditis
 Addison disease 14
 PATHOLOGICAL TYPES OF GULLAIN-BARRÉ SYNDROME

GBS

AIDP AXONAL MILLER FISHER’S

(DEMYELINATING) PATTERN SYNDROME

AIDP +
AMAN
Secondary
Degeneration
AMSAN
(Acute Motor Sensory
Axonal Neuropathy)
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PIN PERDOSSI, SOLO, 6-8 NOVEMBER 2014
PATHOLOGICAL BASE of GBS

1. Immunopathogenesis

2. Histopathology

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 DISEASE-MODIFYING
FACTORS

Bacterial factors
• LOS biosynthesis cluster
• Ganglioside mimicry of LOS

T cell
Immune Response to Host Factors
LOS • Genetic polymorphism
• immune status

Antigen B cell
Presenting cell

Plasma cell
Cross-reactive
antibodies to nerve
gangliosides

Antibodies
Figure :
Macrophage

Extent of nerve damage

Immunobiology of
Nerve
dysfunction
• Ganglioside distribution in
nerve
• Specificity/affinity antibodies
GBS
• Complement activation

Complement Conduction
dysfunction /
block

Clinical prognostic factors :

Nerve repair  Age
 Severity at onset
 Diarrhoea

Outcome

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 AIDP

• Immunophatogenesis
– Autoimmune Attack  against Antigen in Myelin
(as Cross Reaction to Antigen in Infective Agents)
• Anti-GM1 Antibodies Found in 50% Cases  GM1
Ganglioside antibodies +  Supportive D/ but not required
• Histophatology
– Monophasic Demyelinating (Polyradiculo Neuropathy)

(Barker, et al 2005. pp 336-340)

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 AIDP

• Pathological anatomy : demyelination of

neurites/dendrites
• ENG (Neurophysiology) :
 delayed/prolonged latent periode
 Slow nerve conduction velocity
• Neuroimmunohistology of nerves :

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 Pathogenesis of AMAN

The real pathomechanism of

AMAN has not been definitely
stated.

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 AMAN . . .
• Immunopathogenesis
– Molecular Mimicry of Human Gangliosides by
Bacterial Lipo-oligosaccharida(LOS) of
Campylobacter Jejuni(Carbohydrate Mimicry)
– Antibodies Binding to GM1 or GD1a
gangliosides at nodes of Ranvier  activate
Complement  Disrupt Na-Channel Cluster &
Axoglial Junction  NC Failure  Muscle
weakness
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 AMAN . . .
• Histopathology
–Axonal Degeneration of Neuron
(Neurites)
–Pathological changes at Nodal &
Paranodal Axolemma 
Electrophysiology : Rapidly Reversible
NC Blockade/Slowing (of NCV)

(Lancet Neurol
PIN PERDOSSI, SOLO,.Kuwabara S, Yuki N 2013; 12: 1180-88)23
6-8 NOVEMBER 2014
 AMAN . . .

• ENG (Neurophysiology) :
 Decrease amplitudo of Action Potential
 No compound muscle action potential (CMAP (-))
 INEXCITABLE
• Pathology (Autopsy) : Severe axonal
Degeneration ( (-) demyelination)
• Immunohistology : After episode of C. jejuni
enteritis  of Ig G antibodies against GM 1
(ganglioside M1)
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 CLINICAL SYNDROME of GBS
• Acute onset and Progressive course Weakness of LMN type :
- hyporeflexy or areflexy of peripheral cranial / spinal nerves
• Preceded with paresthesia, 2-3 weeks after episode of fever
• CSF Feature : Albuminocytologic Dissociation
(Normal Cell , ↑ Albumin content)
• Electrophysiology study :
– ↓ NCV and ↑ Distal Latency
_ ↓ Reflex / Absent
• AMAN :
– Pure motor Axonal Subtype
– Onset to 1st Exam < (more rapidly) (<AIDP)
– Onset to PEAK of clinical syndrome  > rapid

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 CLINICAL VARIANTS of GBS
1. Acute Inflammatory Demyelinating
Polyradiculoneuropathy (~ AIDP)
2. Subacute Inflammatory Demyelinating
Polyradiculoneuropathy
3. Acute Motor Axonal Neuropathy (AMAN)
4. Acute Motor Sensory Axonal Neuropathy
(AMSAN)
5. Acute Sensory Ataxic Neuropathy (ASAN)
6. Miller-Fisher’s Syndrome
7. Acute Pan-Autonomic Neuropathy

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 OTHER ACUTE NEUROPATHIES , include :
• LIME DISEASE
• PHORPHYRYA
• VASCULITIS and NON-SYSTEMIC VASCULITIS NEUROPATHY
(esp. SLE)
• HIV DISEASE
• PARANEOPLASTIC NEUROPATHY
• LEAD POISONING

 POLIOMYELITIS
 BOTULISM
 BRAINSTEM STROKE
 HYSTERIA
Barker,et al, 2005
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•Korinthinberg R, Schessl J, Kirschner J. Clinical presentation and course of childhood Guillain-Barre
PIN PERDOSSI,
syndrome: a prospective multicentre study. SOLO, 6-8 NOVEMBER
Neuropediatrics. 2014
2007;38:10-17. 29
Tabel 2. CLINICAL FEATURES OF THE MAJOR SUBTYPES OF GULLAIN-BARRÉ SYNDROME

Lancet Neurol
Kuwabara S, Yuki N 2013; 12: 1180-88
PIN PERDOSSI, SOLO, 6-8 NOVEMBER 2014 30
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 ACUTE ONSET and FAST PROGRESSIVE WEAKNESS (OF MUSCLES)
 LASTING UP TO 4 WEEKS (IF > 4 WEEKS  SUBACUTE / CIDP ?)
 RELATIVELY SYMMETRICAL
 MILD SENSORY DISTURBANCE (DISTAL HYPESTHESIAS/PARESTHESIAS)
 CRANIAL Nn : USUALLY FACIAL (VII),OCULAR,or BULBAR (NERVI IX,X,XII)

 RECOVERY STARTS FROM 2nd - 4th WEEK AFTER PROGRESSIVITY STOPS

 SOMETIMES AUTONOMIC DYSFUNCTIONS : TACHYCARDIA, ARRHTHMIA,
POSTURAL HYPOTENSION, HIPERTENSION or VASOMOTOR SYMPTOMS

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 CSF ABNORMALITIES SUPPORTING DIAGNOSIS
• DISSOCIATION ALBUMINOCYTOLOGIQUE : ELEVATED PROTEIN,N CELL
• (VARIANT) : > 1 WEEK, CELL : 11-50/mm3 ;
Protein : Normal

BLOOD
• UNREMARKABLE, EVIDENCE OF INFECTION
(COMPILOBACTER SEROLOGY)
• ANTI-GANGLIOSIDE ANTIBODIES (MORE RELEVANCE IN
VARIANTS (MILLER FISHER : GQ16 ; ASAN : GD16)

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 • DECREASED NCV (SLOWING
AIDP OF NCV OR BLOCKING (IN
VERY EARLY STUDY : MAY BE
(DEMYELIN NO ABNORMALITY SEEN )
ATION) • ABSENT OR DECREASE F-
WAVE

AMAN
AXONAL • LOW AMPLITUDE OF
DEGENERATI ACTION POTENTIAL
ON
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 TREATMENT
• Immunomediated Neurol Disorders  Thus R/
Immunomodulatory Therapy *^
• AIDP ** : R/ - IVIG, or Immune Modulatory
- Plasma Exchange Therapy *

• CIDP : R/ - IVIG, or **
- Corticosteroids
+ Rehabilitation : ▪ Physical Therapy
▪ Occupational Therapy
▪ Speech Therapy ,when Bulbar Symptoms (+)
• AMAN : Complement Inhibitors (partic : severe AMAN)
* Barker RA, et al (2005)
^ Raphael JC, et al. Cochrane Data Base Syst Rev 2022;2:CD001798
** Misulis KE & Heri TC : Elsevier Saunders, Philadelphia, Netter’s Concise Neurology 2007,pp. 297,336)
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Treatment....

• Immunomodulation via :
• PLASMAPHERESIS, and
• INTRAVENOUS IMMUNOGLOBULIN (IVIg)
have HASTEN RECOVERY in AIDP

NEITHER MODALITY IS CLEARLY SUPERIOR TO

THE OTHER
(Kershen J and Sabin, 2007; pp 1127-1140)
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Treatment..........
• In ICU/ITU
• Plasma exchange
• With IVIg 0,4 g/kg/day for 5 days
• Oral/iv Steroids probably not helpful ,but
this is currently being re-evaluated

(Barker RA, et al : 2005 ; 392-394 )

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TREATMENT........

• Since GBS is an acute and progressive course

disease, treatment should likely to serve in the
ICU, ITU or Emergency Room
• Oxygen tube/masker,
• Tracheal Intubation,
• Nasogastric tube feeding,
• Even tracheostomy ,
when necessary, should be applied.
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 • SURVIVE / RECOVER
± 90 % COMPLETELY

• PERMANENTLY DISABLE
10 % (REQUIRE AIDS)

5% • DIE
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1. Guillain-Barre Syndrome(GBS) is not
uncommon disease; an acute
symmetrical motor(sometimes with
sensory) dysfunction basically due to
immunopathogenetic mechanism with
good prognosis when recognised early
and treated promptly based on its
primary pathomechanism

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2. The diagnosis of GBS
is determined on :
• clinical symptoms of acute onset of
symmetrical weakness, associated with
hypo-/areflexy;
• albumino-cytologic dissociation in the CSF; and
• electro- physiological findings of demyelination
and/or axonal degeneration evidences of
several peripheral nerves.

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3. The treatment of GBS consists of :
immunomodulation in the acute
phase,with :
plasma exchange (plasmapheresis),
intravenous immunoglobulin (IvIg), and
medical rehabilitation with physical therapy.

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4. PROGNOSIS of the GBS
is generally good , unless
late diagnosis and inappropriate treatment.
Most of the GBS patients survived and fully
recovered.

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