CLINICAL ASPECT OF GUILLAIN

BARRE SYNDROME
Alwi Shahab
Bagian Neurologi FK Unsri
RSMH Palembang

Landry

History of GBS
Landry (1859) first described a case of a febrile
illness followed by sensory and motor symptoms
with subsequent respiratory failure and death.
Autopsy unrevealing. Peripheral nerves probably
not examined
The disease was named Landrys Ascending
Paralysis

History of GBS
Guillain, Barre and Strohl
(1916) further
described the disease when two soldiers in
Amiens developed paralysis and loss of deep
tendon reflexes
A new diagnostic feature: albuminocytologic
dissociation in the CSF

INTRODUCTION
Group of immune mediated disorder targeting
the peripheral nerves
Unknown etiology
An acute autoimmune polyneuropathy
encompassing heterogenous group of pathologic
and clinical entities
Various antecedent infections
Post infective acute polyradiculoneuropathy

Cont.
Incidence : 1-3/100,000 population
Both sexes especially male
Any age group especially young adult and the
elderly
Progressive symmetrical weakness and areflexia
Mortality rate : 4-15%
Persistent disability : 20%

SGB SUBTYPE
AIDP

: Acute Inflammatory Demyelinating

Polyneuropathy
AMAN : Acute Motor Axonal Neuropathy
AMSAN : Acute Motor Sensory Axonal
Neuropathy
Miller Fisher Syndrome

CLINICAL FEATURES OF AIDP

2/3 have identifiable preceding event
Progressive for days to weeks
Mostly begin with paresthesia and followed by
weakness on legs ascending to arms
Partial opthalmoplegia happens in 5% of cases;
total opthalmoplegia for 5% of cases as well
Autonomic dysfunction accounts for 65% of
cases

CLINICAL FEATURES OF AMAN

Progressive in similar manner to AIDP

Flaccid paralysis
No sensory dysfunction
Often preceeded by diarrhoea
Similar prognosis to AIDP
Mortality rate of <5%

CLINICAL FEATURES OF AMSAN

Commonly preceeded by diarrhoea
Abrupt onset of weakness quadriplegia
respiratory insufficiency
Longer period of recovery
More residual symptoms
Mortality rate of 10-15%

CLINICAL FEATURES OF MF SYNDROME

LABORATORY FINDINGS
CSF :
- Protein : normal during early phase
elevated after 2 weeks
- Cell count < 10
Antibodies :
- AIDP : Non spesific
- AMAN : IgG anti GM1
- AMSAN
: IgG anti GD1a
- MFS : IgG anti GD1b

ELECTROPHYSIOLOGY FINDING
AIDP
:
- prolonged F & distal motor latencies
- conduction block
AMAN :
- reduced CMAP
- normal F & distal motor latency
- normal sensory studies

Cont.
AMSAN :
- no response in some motor nerve
- decreased amplitude of CMAP
- fibrillation
- absence of SNAP
MFS
:
- reduced sensory & motor NAP
- absence of H reflex
- normal motor & sensory conduction velocity

DIAGNOSTIC
Clinically
:
- rapidly progressive ascending symmetric
paralysis
- areflexia
- antecedent infection history (mostly)
Laboratory
:
CSF : cyto-albuminique dissociation
especially after 2nd week from onset

Cont.
EMG
AIDP:

- evidence of demyelination on CNS

- absence of F wave
AMAN:
- reduced motor amplitude
- normal sensory study
AMSAN:
- reduced motor and sensory amplitude
MFS:
- decreased of sensory NAP

DIFFERENTIAL DIAGNOSIS

Acute spinal cord diseases

Acute neuromuscular junction disorder
Acute metabolic myopathy
Neuropathy

TREATMENT
Plasma exchange
- 50ml/kgBW 3-5times over 1-2 weeks
- limited, invasive, serious side effects
IVIg
- 0,4gr/kgBW/day over 5 days
- less invasive, easy administration, less
serious side effects
Respiratory care
Physical therapy

Cont.
Supportive therapy
- DVT prophylaxis
- Psychiatric consult for reactive depression
Nutritional support
Pain management

Case illustration
A 39 year old male was admitted with chief
complaint of progressive quadriplegia starting 2
days ago. Initially the patient felt painful and
tingling sensation on both lower limbs which
later ascended to the upper limbs. Patient was
still able to walk. A day after all the symptoms
worsen and the patient was not able to walk
anymore. 2 weeks before, the patient suffered
from varicella. No history of spinal trauma or
chronic cough.

Physical examination
General status :
Compos mentis
BP : 120/80mmHg
HR : 78x

RR : 20x
T : 36.8 C

Neurological status :
Cranial nerve functions : in normal limit
Motor function
Left upper limb

Right lower limb

Movement

Right upper
limb
Inadequate

Inadequate

Inadequate

Left lower
limb
Inadequate

Strength

Tone

Clonus
Physiiological
reflex
Pathological reflex

Sensory function
: paresthesia on tip of four limbs
Higher cortical function : in normal limit
Autonomic function
: in normal limit
Meningeal reflexes
:Gait and balancing
: steppage gait
Abnormal movement : -

Diagnosis : Flaccid quadriplegia e.c suspected Guillain

Barre Syndrome
Radiology findings :
Chest X-ray : in normal limit

Laboratory findings :
- Hb : 14,6 mg/dl
- Leu : 12,700 mm3
- Diff. Count : 0/01/79/13/7
- Glucose : 120 md/dl
- Total cholesterol : 228 mg/dl
- HDL : 39 mg/dl
- LDL : 139 mg/dl
- Triglycerides : 258 mg/dl

- Ureum : 42 mg/dl
- Creatinine : 0,9 mg/dl
- Uric acid : 1,8
- Natrium : 145 mmol/L
- Potassium : 4,1 mmol/L
- Calsium : 1,96 mmol/L
- Widal O titer : 1/80
- Widal H titer : 1/80
- ASTO : (-)
- CRP : (+)

Dermatology and venerology consult :

Varicella in recovery process + hyperpigmentation

Day 1 :
Compos mentis
BP : 120/80 mmHg, HR : 78x/min
Spontaneous breathing, reguler, adequate, RR : 20x/min
Strength of upper limb 3, lower limb 4
Tingling sensation on the tip of hands and feet
Given neurotonic and roborantia
Day 2 :
BP : 140/90 mmHg, HR : 84x/min RR : 20x/min
Strength of upper limb 3, lower limb 2
Day 3 :
Somnolence
BP : 170/110 mmHg, HR : 102x/min
Dyspnoe, RR : 28x/min
Strength of upper limb 1, lower limb 1
Moved to ICU

Day 6 :
Spontaneous respitarory (-)
BP : 143/98 mmHg, HR : 85x/min
Ventilator is used
Administration of IVIG 400 mg/kg BW/day for 5 days
Day 9 :
BP : 181/113 mmHg, HR : 118 x/mnt
Day 10 :
Compos mentis
BP : 98/60 mmHg, HR : 117x/min
Last day of IVIG
Tracheostomy
Strength of upper limb 2, lower limb 1
Hari 17 :
BP: 140/85 mmHg, HR: 112x/min.
Spontaneous respiratory (+), RR : 40x/min.

Day 19 :
BP : 147/85 mmHg, N: 103x/min
Spontaneous respiratory (+)RR : 26x/min.
Strength of upper limb 2, lower limb 2.
Day 29 :
BP : 130/90 mmHg, RR : 98 x/min
Spontaneous respiratory (+), adequate, RR : 22x/min
Moved to regular room
Strength of upper limb 3, lower limb 1
Day 37 :
BP:120/80 mmHg, HR: 86x/min
Strength of upper limb 3 lower limb 2

Day 53 :
BP : 120/80 mmHg, HR: 82x/min
Strength of upper limb 4, lower limb 3
Day 69 :
BP : 120/90 mmHg, HR : 78x/min
Strength of upper limb 4, lower limb 4
Tracheostomy off
Patient was sent home at day 72 with strength of
upper limb 4 and lower limb 4.

THANK YOU