The document describes a case of acute flaccid paralysis (AFP) in a 12-year-old male who presented with pain and weakness in his lower limbs. On examination, he had hypotonia, hyporeflexia, and reduced motor strength in both upper and lower limbs. Guillain-Barré syndrome (GBS) is the most common cause of AFP and involves demyelination of peripheral nerves due to autoimmune attack. Key diagnostic features of GBS are rapid onset of symmetric ascending paralysis, intact sensation, absence of fever or cranial nerve involvement, and albuminocytologic dissociation on CSF analysis. Treatment involves hospitalization, IV immunoglobulin or plasmapher

1. ACUTEFLACCIDPARALYSIS
(AFP)
Prepared by;- Dr. SafeerAhmed Jamil
RESIDENT FCPS II TRAINEE
DEPTT. OF PEDIARICS

2. CASE
• A 12 yr old male,came to ED with complaints of pain in the calf
of the right leg on Tuesday,followed by weakness of the both
lower limbs the next day morning.was bed ridden after the
onset of weakness.his bowel and bladder are intact.he is able to
feel sensation of clothes over the lower limbs.he felt heaviness
of the limbs..taken to local hospital was given
treatment..where he noticed weakness in the upper limbs also.
• no symptoms of cranial nerve involvement.
• no h/o fever at the onset of weakness.
• no h/o recent immunization or no h/o dog bite.
• no h/o seizures episodes.
• no h/o similar complaints in the past.
• no h/o similar complaints in the family.

3. Clinical examination
• Patient was consciuos coherent
• no pallor ,icterus,clubbing,lymphadenopathy,edema.
• His BP is 110/70 mm Hg in supine,100/70 mm Hg on
standing.no postural hypotension.
• Pulse -82/min regular,all peripheral pulses felt.
• Respiratory rate -18 /min.breath holding time –able to
count upto 45 in single breath.

4. NERVOUS SYSTEM
• Higher intellectual functions intact
• Cranial nerves normal
• Motor system showed hypotonia of all four limbs.
• Grade 3/5 in upperlimbs,2/5 across the hip joint,knee
joint,o/5 power across ankle joint.
• Hyporeflexia all DTRs,absent ankle
• Foot drop b/l
• Babinski negative,abdominal present
• Sensory intact
• Gait –stance narrow based
• Buckling of knees while walking.
• Waddling gait..high stepping gait
• Other systems normal.

5. Key points
• Rapid progressive ascending paralysis.
• Acute onset
• Motor paralysis
• Intact sensory
• No cranial nerve involvement
• No bladder involvement
• No fever at onset of weakness

6. Diagnosis………………??????????????????

7. DEFINITION
• 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 .

8. Guillain-Barré Syndrome.
 Poliomyelitis.
Hypokalemic periodic paralysis
Transverse Myelitis.
Botulinum toxicity
DD of Acute Flaccid Paralysis

9. Involvement
• AHC:
1- acute poliomyelitis
2- acute transverse myelitis
• Peripheral Nerves:
1- roots: GBS (post-infectious)
2- toxins: Diphteria, porphyria

10. Involvement
• N-M junction:
botulinum toxin
tick toxin
• Metabolic:
Periodic paralysis
• Muscular:
myositis

11. Climbing
Paralysis

12. • 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.
Guillain-Barré Syndrome :

13. Guillain Barre Syndrome GBS
• The most common cause of acute flaccid
paralysis (AFP) among infants.
• Age : any including newborn
• Sex : any ( male > female)
• Post-infectious polyneuropathy; ascending
polyneuropathic paralysis
• An acute, rapidly progressing and potentially
fatal form of polyneuritis

14. Pathophysiology
Autoimmune disorder
(T cell sensitization) causes
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

15. pathophysiology
• 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

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

18. Preceding Events : (1-3 WEEKS)
• Respiratory infections :
1- Viral: CMV, EBV, Varicella virus , influenza
virus
2- Bacterial: Mycoplasma pneumoniae, H
influenza
• Gastrointestinal infections :
Campylobacter-jejuni (Bloody GE)
• Vaccinations
• Post surgery

19. Aetiology
▫ Mycoplasma
•Hepatitis B
•CMV
•EBV
•Measles
•Mumps
•Echovirus
•Cocksakie virus
•Influenza virus
▫ Varicella virus
•Compylobacter jejuni

20. Usually 2 - 4 weeks following respiratory or GI infection.
• The classic presentation:
* Fine paresthesias in the toes and fingertips.
* Lower extremity weakness: symmetric &
ascending(landry ascending paralysis).
* 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.
Clinical Features of GBS :

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

22. Guillain-Barre’ Syndrome
• Affects the peripheral nervous system

23. Characteristic “3A”triad:
• 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)

24. Serious Association:
• respiratory failure:
▫ diaphragmatic weakness (Phrenic nerves.)
▫ respiratory muscles weakness
▫ oropharyngeal weakness: impaired swallowing of
secretions & aspiration
• cardiac arrest
• aspiration
pneumonia

25. • 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.
Forms of GBS :

26. CLINICAL VARIANTS
1–Polyneuritis cranialis
Cranial nerve involvement
2–Miller fisher syndrome
Ophthalmoplegia, ataxia, areflexia
3–Chronic progressive GBS
Symptoms persisting more than 6 weeks
4- Chronic relapsing GB

27. Differentiation from spinal cord
syndrome
• Loss of arm reflexes
• Absence of sensory level
• Lack of spinal tenderness
• Normal bowel and bladder function

28. GBS IS A CLINICAL DIASGNOSIS LABS JUST
ASSISST OR RULE OUT OTHER CAUSES OF AFP
Early
Nerve Conduction Velocity (NCV) abnormality
AFTER 1ST
WEEK
• Late : CSF study : albuminocytogenic dissociation

29. 1- NCV/EMG:
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

30. 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

31. 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)

32. 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

33. • 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.
Doubt the Diagnosis of GBS IF:

34. Treatment Hospitalization
• 1- General care
• 2-- Specific treatment
• 3- complication treatment

35. Treatment
• Hospitalization : i- Must be
treated in a hospital, never at home
ii- because of a risk
of sudden onset of cardiac or
respiratory arrest
iii- any hospital ?
No, it must be a hospital have a
pediatrics ICU
Hospitalization is continued until the
child's condition has clearly
stabilized.

36. 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

37. Specific treatment
• i- IV immunoglobin: 2 gm/kg
treatment
*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

38. complication treatment:
i- artificial respiration for
respiratory failure
ii- muscular pain: pain killer as NSAI
iii- chronic relapsing: trial of
immunosupprive drugs or corticosteroid

39. 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

40. 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
mmHg)
- 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.

41. • 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.

42. PROGNOSIS
• Mortality 3%
• 20% of cases need repiratory ve
• Recovery
- 1 to 6 months, may take 12 months
- Delayed recovery may be followed by
permanent neurological sequel
Poor prognostic features with sequelae if at
presentation.
1. Cranial nerve involvement
2. Needs Intubation & vent.
3. Maximum disability

43. Transverse Myelitis:
• ? of immunological disorder
• C/P : of AFP ( acute onset of flaccid hypotonic
weakness ) with the following characters:
▫ LL paralysis : paraplegia with areflexia
▫ with sensory level of loss of sensation
▫ Later : hyperreflexia

44. 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

45. 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.