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sudden onset of weakness or paralysis

over a period of 15 days in a patient aged
less than 15 years age.

1- acute poliomyelitis
2- acute transverse myelitis

Peripheral Nerves:
1- roots: GBS (post-infectious)
2- toxins: Diphteria, porphyria

N-M junction:
botulinum toxin
tick toxin

Periodic paralysis

Case Presentation
Guillain Barre Syndrome GBS
The most common cause of acute flaccid
paralysis (AFP) among infants.

Age : any including newborn

Sex : any ( male > female)
Guillain-Barre Syndrome
Post-infectious polyneuropathy; ascending
polyneuropathic paralysis

An acute, rapidly progressing and

potentially fatal form of polyneuritis
Autoimmune disorder (T cell
MYELIN SHEATH cause of demyelination
Due to attack of the myelin sheath of
nerves by:
antibodies (Ig M, Ig G)
white blood cells (macrophages)
Complement activation on the outer
surface of myelinated fibers
Because (POST-)
Virus/Bacteria share antigenic sites with
axons & peripheral nerve sheath or both
inflammation causes leakage of proteins
into the CSF causing raised CSF proteins
without pleocytosis
Can involve the peripheral nerves, cranial
nerves,dorsal roots, dorsal root ganglia &
sympathatic chain
Preceding Events : (1-3 WEEKS)
Respiratory infections :
1- Viral: CMV, EBV, Varicella virus , influenza
2- Bacterial: Mycoplasma pneumoniae, H influenza

Gastrointestinal infections : Campylobacter-

jejuni (Bloody GE)

Post surgery
Hepatitis B
Cocksakie virus
Influenza virus
Varicella virus
Compylobacter jejuni
classification of GBS
(Clinical, Pathological & neurophysiological)

1-Classic type (mixed): Acute inflammatory

demyelinating polyradiculo-neuropathy (AIDP)
2- Pure motor GBS*
3- Pure sensory GBS
4- Pure pandysautonomia
5-- Miller-Fisher syndrome ( hypotonia, ophthalmoplegia,

NB., Pure Motor GBS*

Usually Post Campylobacter-.jejuni infection

1- IP ( afrebrile ) : 1-3 weeks

2- CP: motor , sensory , autonomic
3- Serious Association
Guillain-Barre Syndrome
Affects the peripheral nervous system

Symptoms and Signs

(Typical GBS)

Motor : 1- Symmetric acute progressive ascending weakness <4 wks,

starting in LL
2- Areflexia or hyporeflexia
3- Atonia or hypotonia
Symptoms and Signs
(Typical GBS)

Sensation : 1- C/O pain as hyperthesia or cramps

2- O/E loss of pain sensation (hypothesia) in feet/hands
3- Both C/O, and O/E
Symptoms and Signs
(Typical GBS)

Autonomic :
1- BP: orthostatic hypotension,
labile hypertension
2- bradycardia, arrythmia
3- atonic bladder,
4- constipation
5- flashing and/or sweating
6- alteration of temperature
Characteristic 3Atriad:

ascending weakness : a- bilateral symmetrical weakness

b- usually start in LL, then UL
c-then, might be affected :
i- cranial nerves (Brain stem) :
including glosssopharyngeal and vagus nerves ( difficulty of swallowing
even of fluid and water) and III, IV, VI cranial nerves ( eye muscles in
Miller Fisher variety), VII Facial nerve ( unilateral or bilateral), and then
respiratory muscles
ii- respiratory muscles
iii- phrenic nerves ( diaphragm )
areflexia ( Hallmark)
atonia ( hypotonia)
Serious Association:
respiratory failure:
diaphragmatic weakness (Phrenic nerves.)
respiratory muscles weakness
oropharyngeal weakness: impaired swallowing of
secretions & aspiration
cardiac arrest
aspiration pneumonia

1Polyneuritis cranialis
Cranial nerve involvement
2Miller fisher syndrome
Ophthalmoplegia, ataxia, areflexia
3Chronic progressive GBS
Symptoms persisting more than 6 weeks
4- Chronic relapsing GB
Differentiation from spinal cord
Loss of arm reflexes

Absence of sensory level

Lack of spinal tenderness

Normal bowel and bladder function


Nerve Conduction Velocity (NCV) abnormality


Late : CSF study : albuminocytogenic dissociation

i- Early :Delayed or absent F

waves or H reflexes
ii- slow or block of Nerve
conduction velocity
iii- normal EMG/ extensive
fibrillation showing denervation
2- CSF: Albuminocytologic
dissociation (Froin Syndrome)

i- Increased CSF protein with

normal cells
ii- might be normal CSF
during 1st week
ii- usually +ve after 2 weeks
of onset

Differential Diagnosis of
cytoalbuminous dissociation
1- GBS
2- poliomyelitis
3- Diphteria polyneuritis
4- spinal cord compression
5- transverse myelitis
6- infratentorial tumor
7- venous sinus thrombosis
8- lead poison
9- botulism
Investigations (OTHERS)

Antibody study : Ig M autoantibodies to GM1 and GM2

gangliosides or Spinal MRI or normal CPK
Anti-GQ1b antibodies are typically found in patients with the
Miller Fisher syndrome (Acta Pediatr 2011)
Outcome of GBS patients

Regressive : 90 % of patients making a good recovery,

after 2-3 weeks of onset, Recovery, usually beginning 2 to
4 weeks after progression stops starting from the last
muscles affected till lower limb ( descending pattern )
Chronic Relapsing: Less than 5% of patients
Mortality: 3-% die from complications as respiratory
failure especially in infants
1- General care
2-- Specific treatment
3- complication treatment
Hospitalization : i- Must be treated in a
hospital, never at home
ii- because of a risk of
sudden onset of cardiac or respiratory
iii- any hospital ? No, it
must be a hospital have a pediatrics ICU

Hospitalization is continued until the child's

condition has clearly stabilized.
General care
i- bed sores
ii-bowel care
iii- nutrition care
monitoring of vital signs
Nursing care
Repeated spirometries
Bowel and bladder care
Tube feeding
Care for bed sores
Ventilatory support if required
Specific treatment

i- IV immunoglobin: 2 gm/kg
*at a dose of 0.4 g/kg/day for 5
consecutive days or
* 1gm/kg/day for 2 days
ii- plasmapheresis: 5 exchanges
of 50 ml plasma/ kg on alternate days (
10 days course).
iii- both i and ii
complication treatment:

i- artificial respiration for

respiratory failure
ii- muscular pain: pain killer as
iii- chronic relapsing: trial of
immunosupprive drugs or
Need for intensive care (PICU)

- Flaccid quadriparesis
- Rapidly progressive weakness
- Reduced vital capacity (20 mL/kg)
- Bulbar palsy
- Autonomic cardiovascular instability

Need for assisted ventilation Approximately 20

percent of children with GBS require mechanical
ventilation for respiratory failure
Warning signs for RF*
- A sustained increase of pCO2 to 50 mmHg (normally
35 to 40 mmHg)
- An increasing respiratory rate
- Increasing oxygen requirement and increasing
alveolar to arterial oxygen difference (normally 5 to 10
- An increased use of accessory muscles (eg,
sternocleidomastoid use, flaring of the ala nasae,
intercostal retractions) and decreased or paradoxical
diaphragm movements; these reflect restrictive lung-
chest wall movement and low lung volumes
- Sweating about the head and neck, wide pulse
pressure, and bounding pulses portend CO2 retention.
Children have less metabolic and muscle reserve
than adults. They can deteriorate quite rapidly and
become apneic or develop alveolar hypoventilation
"right under your nose."
Sedation and neuromuscular blockade should be
avoided in ventilated patients because they
obscure the course of the illness.
Providing scrupulous airway care and chest
physiotherapy reduce the risk of pneumonia.
Tracheostomy may need to be performed if
prolonged ventilation is required.

Mortality 3%
20% of cases need repiratory ve
- 1 to 6 months, may take 12 months
- Delayed recovery may be followed by
permanent neurological sequel
Transverse Myelitis:

? of immunological disorder
C/P : of AFP ( acute onset of flaccid
hypotonic weakness ) with the following
LL paralysis : paraplegia with areflexia
with sensory level of loss of sensation
Later : hyperreflexia
Poliomyelitis :

due to enterovirus affection (polio virus ) ( non or

inadequate OPV )
C/P: of AFP with the following characters:
first shock stage ( with generalized hypotonia) then
patchy asymmetrical weakness
normal sensation
areflexia of affected muscles