1) Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy that is a common cause of acute flaccid paralysis. 2) It is caused by an autoimmune attack against peripheral nerves following a viral or bacterial infection. Campylobacter jejuni is a frequent trigger that shares antigens with human nerves. 3) Clinical features include ascending limb weakness, diminished reflexes, and possible involvement of cranial nerves and respiratory muscles. Diagnosis involves CSF analysis showing elevated proteins with normal cell count and electrophysiology showing demyelination.

1. Acute Flaccid Paralysis
(AFP)
Dr . AJAYAN.P
JIPMER

2. digram of a cross section through the spinal cord
The dorsal root on each side conveys sensory information to the spinal cord;
the ventral root conveys motor commands to the muscles.

3. Differential Diagnosis of Acute Weakness:
 Cerebral: Bilateral strokes, Hysteria…
 Cerebellar : Acute cerebellar ataxia syndromes.
 Spinal: Compressive myolopathy ,
Transverse myelitis .
 Peripheral nerve: Acute inflammatory demyelinating
neuropathy,
Toxic neuropathy,
Diphtheria,
Tick paralysis

4. DD of Acute Weakness (cont):
Neuromuscular junction:
Botulism,
Myasthenia Gravis…
Muscle disease: Acute myositis.
Acute inflammatory myopathies
Metabolic myopathies ,
Periodic paralysis…

5. Acute Flaccid Paralysis

6. Acute Flaccid Paralysis
WHO defines AFP syndrome as “ Rapid onset of
weakness of an individual’s extremities, often
including weakness of muscles of respiration &
swallowing, progressing to maximum severity
within 1-10 days.
The term ‘flaccid’ indicates the absence of :
 Spasticity.
 Other signs of disordered CNS motor tracts such
as :
Hyperflexia,
Clonus,
Extensor plantar response .

7. Clinically spasticity is defined as
velocity dependent resistance to
stretch, where a lack of inhibition
results in excessive contraction of the
muscles, ultimately leading to
hyperflexia (overly flexed joints).

8. AFP is an emergency in which
management priorities are to
support vital functions and reach
a specific diagnosis in a timely
manner with a focused history
and physical examination.

9. DD of Acute Flaccid Paralysis
Guillain-Barré Syndrome.
 Poliomyelitis.
Transverse Myelitis.

10. Fever at onset - Polio or enteroviralmyelitis,
Transverse
myelitis, myositis, epidural abscess, and
Koch spine (prolonged history)
Trauma: head/neck Trivial trauma may lead to
spinal
compression in patients with cervical
vertebral instability (Patients with
Downs syndrome, congenital
cervicovertebral anomalies or juvenile
idiopathic arthritis)

11. Exposure Toxins: lead, arsenic
Snake envenomation
Dog bite: Rabies
Preceding infectious
prodrome/vaccination
Guillain Barre syndrome or transverse
myelitis
Sore throat, neck swelling- diphtheretic
polyneuropathy (non/partly immunized

12. Precipitating factors Diarrhea: Hypokalemia,
enteroviral
myelitis
Exertion or post parandial: Hypokalemic
periodic paralysis
Intramuscular injection: Polio, traumatic
sciatic neuritis
Sensory loss/level -Compressive myelopathy,
transverse
myelitis

13. Early bowel/bladder
involvement
Compressive myelopathy, transverse
myelitis
Constipation in <1 y Botulism (H/o honey
exposure)
Prominent autonomic
signs/symptoms
Guillain Barre syndrome, Rabies, acute
myelopathy

14. Ascending weakness Guillain Barre syndrome,
Rabies,
Varicella zoster virus, ascending myelitis
Descending weakness ---Diphtheria, Botulism
Prominent and earl
ptosis
Myasthenia Gravis, Botulism

15. Facial weakness Guillain Barre syndrome,
Myasthenia
Gravis, Botulism
Fluctuating symptoms,
fatigability
Myasthenia Gravis
Muscle tenderness ----Myositis, inflammatory
myopathy,
(myalgias may be severe in Guillain
Barre syndrome

16. Muscle stretch reflexes
Absent: Guillain Barre syndrome, Polio,
Diphtheria, spinal shock, at level of
spinal cord damage
Preserved : Myasthenia Gravis, periodic
paralysis, Botulism
Exaggerated : Below level of spinal lesion,
Upper motor neuron lesion

17. Spinal tenderness,
painful spine
movement
Spinal trauma, epidural abscess

18. 1)Spinal cord -Compressive -Traumatic spinal
injury, epidural
abscess, hematoma, discitis
inflammatory-- Transverse myelitis

19. 2)Anterior horn
cell
Viral - Poliomyelitis, vaccine associated
poliomyelitis, Enteroviral
myelitis, Japanese encephalitis
Vascular ----Anterior spinal artery infarction

20. 3)Roots/nerves- Immune mediated-- Guillain
Barre syndrome,
Toxin-- Post diphtheritic, porphyria,
arsenic
Viral ------Rabies
Trauma ----Injection related sciatic neuritis

21. 4)Neuromuscular
junction
Immune mediated--- Myasthenia Gravis
Drugs, toxins-- Organophosphates, snake venom,
drugs (aminoglycosides),
Botulism
Dyselectrolytemia ------Hypermagnesemia
5

22. 5) Muscle ----Infection Viral myositis
Inflammation --nflammatory myopathy
(polymyositis)
Channelopathy ---Hypokalemic periodic
paralysis
Dyselectrolytemia -------------------Hypokalemia

23. Guillain-Barré Syndrome

24. Guillain-Barré Syndrome :
• It is an acute idiopathic monophasic acquired
inflammatory demyelinating polyradiculo-neuropathy.
• Polyradiculopathy refers to damage to multiple
nerve roots sufficient to produce neurologic
symptoms & signs such as pain, weakness, and
sensory affection.
• GBS is the most common cause of acute flaccid
paralysis in healthy infants and children.

25. )1865-1826(
Georges Charles Guillain,
neurologist (1876-1961),
André Strohl,
physicien (1887-
1977)
Jean-Alexandre Barré,
neurologist (1880-
1967)
French neurologists and physicians first started
describing Guillain-Barré Syndrome in 1859

26. Epidemiology :
• The annual incidence 0.6 to 2.4 cases / 100,000
population and occurs at all ages and in both sexes.
• The incidence is lower in children, 0.38 and 0.91
cases / 100,000 in two reports.
• Occurs rarely in children younger than 2 years of
age, but can occur in infants.
• Males are affected 1.5 times <females in all ages.

27. GBS.Pathophysiology :
• Immune mediated disease.
• There is no known genetic factors.
• 2/3 of cases follow a respiratory or GI infection.
• Campylobacter infection is the most common, but other
organisms include CMV, EBV, HSV, Enteroviruses,…
• Guillain-Barré syndrome has been reported to follow :
– vaccinations .
– epidural anesthesia.
– thrombolytic agents.

28. The pathophysiologic mechanism of an antecedent
illness and of GBS can be typified by Campylobacter jejuni
infections.
 The virulence of C jejuni is thought to be based on the
presence of specific antigens in its capsule that are shared
with nerves.
Immune responses directed against lipopolysaccharide
antigens in the capsule of C jejuni result in antibodies that
cross-react with ganglioside GM1 in myelin, resulting in the
immunologic damage to the peripheral nervous system.
 This process has been termed molecular mimicry.

29. GBS.Pathophysiology :
• The main lesions are acute inflammatory
demyelinating polyradiculopathy, with acute
axonal degeneration in some cases,
particularly those following campylobacter
infection.
• A variety of auto-antibodies to gangliosides
have been identified especially with axonal
forms of the disease.

30. Pathologic findings in GBS include :
 lymphocytic infiltration of spinal roots and
peripheral nerves (cranial nerves may be involved
as well),
 followed by macrophage-mediated, multifocal
stripping of myelin.
This phenomenon results in defects in the
propagation of electrical nerve impulses, with
eventual absence or profound delay in conduction,
causing flaccid paralysis.
Recovery is typically associated with
remyelination.

32. Hypothetical mechanism of immune response in Guillain-Barré syndrome Inflammatory
infiltrates

33. Clinical Features of GBS :
Usually 2 - 4 weeks following respiratory or GI infection.
• The classic presentation:
* Fine paresthesias in the toes and fingertips.
* Lower extremity weakness: symmetric & ascending.
* Gait unsteadiness.
* Inability to walk.
* Respiratory muscles involvement.
* Neuropathic pain… low back pain.
• Cranial Neuropathy:
Facial nerve is most commonly affected, resulting in
bilateral facial weakness.

34. Clinical Features… cont :
By the peak of the illness, the frequency of
symptoms was as follows:
- 79% had neuropathic pain.
- 60% could not walk .
- 51% had autonomic dysfunction.
- 46% had cranial nerve involvement.
- 24% could not use their arms.
- 13% required mechanical ventilation.

35. 46%
had
cranial
nerve
involve
ment.

36. Physical Examination

37. Physical Examination :
• Symmetric limb weakness.
• diminished or absent reflexes.
• Vibration and position sensation are affected
in 40% of cases.
• Autonomic dysfunction:
* Cardiac dysrhythmias.
* Orthostatic hypotension,
* Hypertension.
* Paralytic ileus .
* Bladder dysfunction.

38.  A reflex is an involuntary and
nearly immediate movement in
response to a stimulus mediated via
the reflex arc.
 reflexes = peripheral problem.
 reflexes = central problem.

39. Human reflexes
:Stretch reflexes
• Jaw jerk reflex: (CN V)
• Biceps reflex (C5, C6)
• Brachio-radialis reflex (C5, C6, C7)
• Extensor digitorum reflex (C6, C7)
• Triceps reflex (C6, C7, C8)
• Patellar reflex or knee-jerk reflex L2, L3, L4)
• Ankle jerk reflex (Achilles reflex) (S1, S2)
While the reflexes above are stimulated mechanically, the
term H-reflex refers to the analogous reflex stimulated
electrically, and Tonic vibration reflex for those stimulated
to vibration.

40. Name Sensory Motor
Pupillary light
reflex
II III
Accommodation
reflex
II III
Jaw jerk reflex V V
Corneal reflex,
also known as the
blink reflex
V VII
Vestibulo-ocular
reflex
VIII III, IV, VI +
Gag reflex IX X

41. Simple Reflex Arc

42. Clinical Course :
• >90% of patients reach the lowest point of their
function within 2 - 4 weeks, with return of
function occurring slowly over the course of
weeks to months.
• The clinical course of GBS in children is shorter
than in adults and recovery is more complete.
• In patients who did not require mechanical
ventilation, the median time to recovery of
independent walking was 43 to 52 days in
children compared to 85 days in adults.

43. Forms of GBS :
• Acute inflammatory demyelinating polyneuropathy
(AIDP): the most common form in developed countries.
• Acute motor axonal neuropathy: more common in
developing countries. More severe with common
respiratory involvement. Strong association with
campylobacter.
• Acute motor-sensory axonal neuropathy.
• Miller Fisher syndrome: triad of external ophthalmo-plegia,
Ataxia, areflexia with muscle weakness.
• Polyneuritis cranialis: associated with CMV infection.

45. Diagnosis :
Cerebrospinal Fluid:
- After the first week of symptoms
typically reveals:
normal pressure,
normal cell count.
elevated proteins (greater than 50 mg/dL)
- Early in the course (less than one
week), protein levels may not yet be
elevated, ( rarely remain persistently
normal) .

46. Diagnosis:
Electro-physiologic studies:
- Most specific and sensitive tests
for diagnosis.
- Evidence evolving multifocal
demyelination.
- A normal study after several days
of symptoms, makes the diagnosis of
Guillain-Barré syndrome unlikely.

48. Doubt the Diagnosis of GBS IF:
• Marked persistent asymmetry of weakness.
• Persistent bladder or bowel dysfunction.
• Bladder or bowel dysfunction at the onset.
• Mononuclear leukocytosis in the CSF > 50.
• Sharp sensory level.
• Pupillary abnormalities are not seen in GBS.

49. GBS Management

50. GBS Management:
• Critical care monitoring for autonomic and
respiratory dysfunction.
• Children with the following should be admitted to
PICU:
a. Flaccid quadriparesis.
b. Rapidly progressive weakness.
c. Reduced vital capacity (≤20 mL/kg)
d. Bulbar palsy.
e. Autonomic cardiovascular instability.
N.B: Sedation and neuromuscular blockade should
be avoided in ventilated patients because they
obscure the course of the illness.

51. GBS Management:
Risk factors for respiratory failure in GBS:
 Cranial nerve involvement.
 Short time from preceding respiratory illness.
 Rapid progression over less than 7 days.
 Elevated CSF protein in the first week.
 Severe weakness: unable to lift elbows above the bed
unable to lift head above the bed
unable to stand.
 20% of children with GBS require mechanical
ventilation for respiratory failure.

52. Special Therapy :
Immune modulatory therapy:
Intravenous Immunoglobulins.
Plasmapheresis.
• Both therapies have been shown to shorten
recovery time by as much 50%.
• Combining plasma exchange and IVIG neither
improved outcomes nor shortened the duration of
illness.

53. INTRAVENOUS
IMMUNE GLOBULIN

54. I V. IMMUNE GLOBULIN :
• IVIG is preferred to plasma exchange in children
because of the relative safety and ease of administration,
although it has not been shown to have better results.
• Randomized trials in severe disease show that IVIG
started within 4 weeks from onset hastens recovery as
much as plasmapheresis.
• Long-term outcome, however, may not be affected.
• Studies have demonstrated that one effect of the IVIG is
to neutralize neuromuscular blocking antibodies.

55. IVIG Regimens :
• Several IVIG regimens have been utilized. One regimen
includes daily IVIG for 5 days at a dose of 0.4 gm/kg
/day, which results in an improvement within a mean of 2
to 3 days after the start of therapy. Other authors use 2
gm/kg of IVIG given as a single dose or 1gm/kg/day for 2
days.
• One study compared the outcome of 0.4 gm/kg/day given
for 3 days versus 6 days. In that study, the 6 days of IVIG
was superior when “time to walking” was used as an
endpoint.
• When comparing treatments of 1gm/kg for 2 days versus
0.4gm/kg over 5 days, no significant difference in the
effectiveness was noted in the 2 treatment regimens.
However, early “relapses” were more frequently observed
in the shorter treatment group.

56. Plasmapheresis :
• Studies in children indicate that
plasmapheresis may decrease the
severity and shorten the duration of
GBS.
• It is most beneficial when started
within 7 days of the onset of symptoms
but is still beneficial in patients treated
up to 30 days after disease onset.

57. Management…cont :
• Corticosteroids are not effective and
not indicated
• Interferon-ß reported to be beneficial
in individual cases, but its safety and
efficacy have not been established in
clinical trials.

58. Prognosis

59. Prognosis :
• In general, the prognosis in affected
children is better than adults.
• Recurrences are uncommon but can
occur in children. Some may have a
chronic progressive course, whereas
others may show recurrences or
relapses.

60. Prognosis :
At long-term follow up, 93% were free
of symptoms, and the remainder were
able to walk unaided.
50% are ambulatory by 6 mo,
70% walk within ayear of onset of the
disease.
Mortality is approximately 3 to 4%,
and usually is secondary to autonomic
dysfunction and respiratory failure.

62. Please don’t miss the diagnosis of GBS. By
noting:
 Symptoms begin 2 - 4 weeks following
respiratory or GI infection.
 Diminished or absent reflexes.
 Symmetric & ascending lower
extremity weakness.
 Sensations intact : Fine paresthesias in
the toes and fingertips.
 No bladder or bowel dysfunction at
the onset.