An Overview of acute flaccid pralysis in Children

1. Acute Flaccid Paralysis (AFP)
BY:
Azad A Haleem
DCH. FIBMS
University of Duhok, College of Medicine
Heevi Pediatrics Teaching Hospital
azad82d@gmail.com
2014

2. Objectives
• Definition of AFP
• Differential Diagnosis of AFP
• SURVEILLANCE
• Protocol for AFP surveillance
• Certification (polio free)
• Epidemiology
• Clinical approach to children with AFP
• How to differentiate among
(Polio, Guillain-Barré syndrome, Traumatic
neuritis, Transverse Myelitis )

3. Definition of AFP
• sudden onset of weakness or paralysis in a
previously normal limb over a period of 15
days in a patient aged less than 15 years age.
• It is a Lower motor neurone lesion

4. Differential Diagnosis of AFP
• SPINAL CORD
• Anterior horn cell disease
• PERIPHERAL NERVE
• Disorders of neuromuscular transmission
• MUSCLE
• Systemic disease

5. SPINAL CORD
• Demyelinating diseases
• transverse myelitis
• Cord compression
• tumour
• trauma
• paraspinal abscess
• haematoma
• vascular malformation with thrombosis/bleeding
• Ischaemic cord damage
• spinal cord stroke
• anterior spinal artery syndrome
• peri-operative complication
Differential Diagnosis of AFP

6. Anterior horn cell disease
• Acute poliomyelitis
• Vaccine-associated paralytic polio
• Other neurotropic viruses (eg. enteroviruses
and herpes viruses)
Differential Diagnosis of AFP

7. PERIPHERAL NERVE
• Unilateral:
• Enteroviral infection
• local trauma
• Focal mononeuropathy
• Bilateral:
• Guillain Barré syndrome
• Acute toxic neuropathies (heavy metals, snake
toxin)
• Neuropathies of infectious diseases (diphtheria)
Differential Diagnosis of AFP

8. Disorders of neuromuscular
transmission
• Myasthenia gravis
• Botulism
• Insecticide (organophosphate poisoning)
• Tick bite paralysis
• Snake bite
Differential Diagnosis of AFP

9. MUSCLE
• Polymyositis
• post viral myositis
• periodic paralysis
• toxic myositis (Corticosteriods and blocking
agents)
• Mitochondrial diseases (infantile type)
Differential Diagnosis of AFP

10. Systemic disease
• Acute porphyrias
• Critical illness neuropathy
• Acute myopathy in ICU patients
Differential Diagnosis of AFP

11. Investigations
• Spinal cord MRI.
• CSF study.
• Nerve conduction study.
• OTHERS

12. SURVEILLANCE
• Surveillance is the collection, analysis,
interpretation and dissemination of
information about a selected health event.
• Health officials use the information to plan,
implement and evaluate health programs and
activities.

13. AFP surveillance in children
• To do this we have to
• report enough cases,
• send stools for enterovirus isolation using a
standardised protocol, and
• follow up children with AFP to determine the
outcome.

14. AFP surveillance in children
• The non-polio AFP rate is an indicator of
surveillance “sensitivity”.
• If it is < 1/100 000 CHILDREN below age 15 per
year then the surveillance system is probably
missing cases of AFP.
• Adequate stool specimens collected from
≥80% of AFP cases.

15. Protocol for AFP surveillance
Step Timing Description
Case Detection at diagnosis Follow case definition for AFP
Case Reporting ≤ 48 hours of report Tel:
Timing of stool
specimens
within 2 weeks of
onset of paralysis
2 stool specimens collected no less than
24 hours apart .
Collection of
specimens
Fresh stool, or rectal swabs containing
fecal material (at least 8g – size of an
adult thumb). Place in a sterile glass
bottle
Transport of
Stools
as soon as able.
Specimens arriving
at national
laboratory ≤ 3 days
of being sent
Maintain a cold chain of 2 - 8 ⁰C.
Transport in dry ice if
transportation will take > 24 hours
Caution: avoid desiccation, leakage;
ensure adequate documentation
Follow up of
patients
60 days from To determine whether there is residual
paralysis on follow up

16. Wild polio cases by country in the region for 2013-2014 (ending 06
April 2014)
2013
14
Afghanistan
93
Pakistan
194
Somalia
26
Syria
327
Total
2014
4
Afghanistan
1
Iraq
43
Pakistan
1
Syria
49
Total
Epidemiology

17. Certification (polio free)
• Basic national documentation has been
accepted from countries that have been polio-
free for 3 years or more.
• Provisional national documentation for
regional certification has been accepted from
countries that have been polio-free for 5 years
or more and have completed phase I of
laboratory containment.

18. • Regular yearly updates (annual update and
abridged annual update) have been
submitted on a yearly basis
• by all countries whose basic national
documentation was accepted (annual update),
• and countries whose final national
documentation for regional certification was
accepted (abridged annual update).

19. Iraq
• Annual updates have been received for years
2002–2006 and accepted.
• Final national documentation for regional
certification has been submitted, and was
accepted in 2008.
• Abridged annual updates and annual updates
have been received and accepted for years
2008–2012.

20. Clinical approach to children with AFP

21. Clinical Questions Demonstrable Lower limb Motor Weakness
Sphincters? Preserved Preserved Preserved/ Affected
Sensory loss? None None ‘Glove & Stocking’ Dermatomal
Reflexes?
Reduced
or normal
Absent Absent, reduced or normal
Clinical
Localisation
MUSCLE PERIPHERAL NERVE SPINAL CORD
Differential
Diagnosis
•post viral
myositis
• periodic
paralysis
• toxic myositis
Unilateral
•enteroviral
infection
• local trauma
Bilateral
• Guillain Barré
syndrome
• toxic
neuropathy
• acute transverse myelitis
• spinal cord / extraspinal tumour
• arteriovenous malformation
• spinal cord stroke
• extradural abscess
• spinal tuberculosis
• spinal arachnoiditis
Investigations
Required
• AFP workup
• creatine kinase
• serum
electrolytes
• urine
myoglobin
Required
• AFP workup
• Nerve
conduction study
Optional
• MRI
Required
• AFP workup
• CSF: cells,
protein
• Nerve
conduction
study
• Forced vital
capacity
Required
• AFP workup
• URGENT Spinal cord MRI
Optional
(as per MRI result)
• TB workup
• CSF : cells, protein,
sugar, culture, TB PCR,
oligoclonal bands

22. How to differentionate among
• Polio
• Guillain-Barré syndrome
• Traumatic neuritis
• Transverse Myelitis

23. Polio
Guillain-Barré
syndrome
Traumatic neuritis
Transverse
myelitis
Installation
of
paralysis
24 to 48 hours onset
to full paralysis
From hours to ten
days
From hours to four
days
from hours to
four days
Fever at
onset
High, always present
at onset of flaccid
paralysis, gone the
following day
Not common
Commonly present
before, during
and after flaccid
paralysis
rarely present
Flaccid
paralysis
Acute, usually
asymmetrical,
principally proximal
Generally acute,
symmetrical and
distal
Asymmetrical,
acute
and affecting only
one limb
acute, lower
limbs,
symmetrical
Muscle
tone
Reduced or absent in
affected limb
Global hypotonia
Reduced or absent
in affected limb
Hypotonia in
lower
limbs
Deep-
tendon
reflexes
Decreased to absent Globally absent
Decreased to
absent
Absent in lower
limbs early
hyperreflexia
late

24. Polio
Guillain-Barré
syndrome
Traumatic neuritis
Transverse
myelitis
Sensation
Severe myalgia,
backache, no
sensory
changes
Cramps, tingling,
hypoanaesthesia of
palms and soles
Pain in gluteus,
hypothermia
Anesthesia of
lower limbs with
sensory level
Cranial nerve
involvement
Only when bulbar
involvement is
present
Often present,
affecting nerves VII,
IX, X, XI, XII
Absent Absent
Respiratory
insufficiency
Only when bulbar
involvement is
present
in severe cases,
enhanced by
bacterial pneumonia
Absent Sometimes
Autonomic
signs
& symptoms
Rare
Frequent
blood pressure
alterations,
sweating and
body temperature
fluctuations
Hypothermia in
affected limb
Present
Cerebro-
spinal
fluid
Inflammatory
Albumin-cytologic
dissociation
Normal
normal or mild
increase in
cells

25. Polio
Guillain-Barré
syndrome
Traumatic
neuritis
Transverse
myelitis
Bladder
dysfunction
Absent Transient Never Present
Nerve
conduction
Velocity
Abnormal: anterior
horn cell disease
(normal during the
first 2 weeks)
Abnormal: slowed
conduction,
decreased motor
amplitudes
Abnormal:
axonal
damage
normal or
abnormal, no
diagnostic value
EMG Abnormal Normal Normal Normal
Sequel at three
months and up
to
a year
Severe,
asymmetrical
atrophy, skeletal
deformities
developing later
Symmetrical
atrophy of distal
muscles
Moderate
atrophy,
only in affected
lower limb
flaccid diplegia
atrophy after
years

26. THANK YOU

27. Guillain-Barré Syndrome
• It is an acute idiopathic acquired
inflammatory demyelinating polyneuropathy.
• GBS is the most common cause of acute
flaccid paralysis in healthy infants and
children.

28. Epidemiology
• It has an annual incidence of 0.6 to 2.4 cases
per 100,000 population and occurs at all ages
and in both sexes.
• Occurs rarely in children younger than two
years of age, but can occur in infants.
• Males are affected approximately 1.5 times
more often than females in all age groups.

29. Pathophysiology
• Immune mediated disease.
• There is no known genetic factors.
• Two third 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

30. Clinical Features
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.
• Cranial Neuropathy:
Facial nerve is most commonly affected, resulting in
bilateral facial weakness.
Miller Fisher syndrome: triad of external
ophthalmoplegia, Ataxia, areflexia with muscle weakness.

31. 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

32. 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

33. Clinical Course
• >90% of patients reach the nadir of their function within two
to four 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.

34. 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
• Polyneuritis cranialis: associated with CMV infection

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

36. GBS Management
• Critical care monitoring
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.

37. 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.

38. Special Therapy
Immune modulatory therapy:
 Intravenous Immunoglobulins
 Plasmapheresis
• 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.
• 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.
• IVIG and plasma exchange are not recommended for
ambulatory children with GBS who have mild disease or
for children whose symptoms have stabilized.

39. 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.

40. Plasmapheresis
• Studies in children indicate that plasmapharesis 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.

41. 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.

42. 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.
• 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.

43. Poliomyelitis
• polio= gray matter
• Myelitis= inflammation of the spinal cord.
• Poliomyelitis is caused by a virus that attacks
the nerve cells of the brain & spinal cord
although not all infections result in sever
injuries and paralysis.

44. Poliomyelitis: Etiology
Etiology:
• Caused by a poliovirus.
• 3 serotypes of poliovirus (genus Enterovirus).
• Type 1 most frequently associated with epidemics.
• Types 2 and 3 usually associated with vaccine-
associated paralytic polio (VAPP).

45. • In 1% of cases virus invades CNS.
• Multiplies and destroys anterior horn cells.
• In severe cases, poliovirus may attacks motor
neurones in brainstem, leading to difficulty in
swallowing, speaking and breathing.

46. Poliovirus: Pathogenesis
• Incubation period of 7 to 14.
• Transmitted by oral-fecal contact.
• Person-to-person spread is the most common means of
transmission, followed by contaminated water.
• During epidemics, it also may be transmitted by
pharyngeal spread.
• Poliovirus initially infects the GI tract. It may spread to
lymph nodes and rarely to CNS.
• The mechanism of spread of poliovirus to the CNS is
not well understood.

47. Epidemiology
• 3 months-16 years; rarely adults
• Predominant sex: Male = Female
• Improved sanitation led to many less infants being
exposed to poliovirus.
• When exposure occurred later and the individuals
were not protected by maternal antibodies, there
were polio epidemics.

48. Poliomyelitis: Incidence & Prevalence
Incidence:
Now rare; present in:
(a) Endemic settings.
(b) Small outbreaks in areas where polio eradication has
occurred.
(c) Rarely as vaccine-associated paralytic polio (VAPP) cases.
Prevalence:
• Endemic countries: Afghanistan, India, Nigeria and Pakistan

49. Poliomyelitis: Risk Factors
• Immune deficiency
• Pregnancy
• Poor sanitation and hygiene
• Poverty
• Unimmunized status, especially if <5 years
• Tonsillectomy: a risk factor for bulbar paralysis.
• Intramuscular injections or truama
Genetics:
• No genetic susceptibility has been identified.

50. Clinical Presentation
• The majority of patients are asymptomatic.
• ~5% develop symptoms.
• ~10% will show signs and symptoms of a minor GI
illness, including fever, malaise, nausea, and vomiting.
• 0.1% develop the paralytic form of poliomyelitis.
• Symptoms of poliomyelitis always CNS specific.

51. Clinical Presentation…cont
CNS manifestations:
• Weakness:
Vary from one muscle or group of muscles, to
quadriplegia.
Proximal muscles: legs more commonly than arms.
Typically worsens over 2 to 3 days but sometimes can
progress for up to a week.
• Bulbar involvement:
5 – 35% producing dysphagia, dysarthria, and difficulty
handling secretions.
• There may be encephalitis, usually in infancy.
• Cardiovascular & Resp. symptoms…bulbar poliomyelitis

52. Physical Exam:
• Significant motor loss on affected side or limb.
• Meningeal signs may be present in minor illness
or early phases of paralytic polio.
• Decreased deep tendon reflexes.
• Muscle atrophy of affected areas.
• Tone is reduced: asymmetric
• The sensory examination is normal.

53. Poliomyelitis: Diagnosis
• Based on the clinical presentation.
• Cerebrospinal Fluid:
Leukocytosis, Increased protein, Normal glucose.
• Virus recovery from stool, throat washing, blood.
 Virus recovery from stool is essential to diagnosis.
 Obtain stool, blood and throat samples for viral serology,
demonstrating a four fold rise in IgG is helpful but not
always easy.
 Positive IgM is diagnostic.
• Polymerase chain reaction amplification of poliovirus
RNA from CSF or serologically, by comparing viral titers
in acute and convalescent sera.

54. Diagnosis…cont
• Electrodiagnostic investigations reveal normal
sensory nerve studies.
• Motor nerve studies:
show normal to mildly slowed conduction
velocities and low to normal amplitudes.
• MRI may be helpful to evaluate involvement of
anterior horn of the spinal cord or other findings.

55. Treatment
• No definitive treatment.
• Mainly supportive: pain relief and physical therapy
for muscle spasms.
• Patients with bulbar involvement require close
monitoring of cardiovascular status and autonomic
dysfunction.
• Mechanical ventilation: Respiratory failure.
• Treatment of complications.

56. Poliomyelitis: Complications
• Urinary tract infection
• Skin ulcers
• Traumatic injuries to affected limb(s)
• Atelectasis & Pneumonia
• Myocarditis
• Postpoliomyelitis progressive muscular atrophy.
• Postpoliomyelitis motor neuron disease.

57. Clinical Course & Outcome
• About two-thirds of patients with acute flaccid paralysis
do not regain full strength.
• The more severe the acute weakness, the greater the
chance of residual deficits, Bulbar squeals are rare.
• The mortality was 5 to 10% in the era of epidemics, and
approached 50% for those with bulbar involvement
because of cardiovascular and respiratory complications.

58. PREVENTION OF POLIOMYELITIS

59. Polio Vaccination
• Jonas Salk created the inactivated poliovirus vaccine
(IPV), using killed virus in 1952.
• The Sabin oral poliovirus vaccine (OPV), using live
attenuated virus, proved successful in 1960.
• In areas of the world where polio is endemic, primary
immunization is still performed with Sabin OPV. But,
because it causes polio in one out of 2.5 million cases, it
has been replaced by the Salk IPV in countries without
polio, including the United States and most of Europe.

60. Polio Vaccination
• Multiple doses required to achieve high humeral
conservation rates against all virus types
• Babies are given 4 doses through out their infancy.
• Adolescents and adults should get vaccinated as well.
• Adolescents younger than 18 should receive the routine
four doses.
• You should get it if you travel outside places where polio
is still epidemic.

61. Transverse Myelitis
• Transverse myelitis is a condition
characterized by rapid development of both
motor and sensory deficits .
Transverse Myelitis

62. Pathophysiology
• Presumed autoimmune mediated
inflammation and demyelination of the spinal
cord.
• Postinfectious etiology largely predominates in
children
• An immunization history within the few weeks
preceding neurologic difficulties.
Transverse Myelitis

63. Classical History
• Mean age of onset is 9 years.
• Symptoms progress rapidly, peaking within 2
days.
• Usually level of myelitis is thoracic.
• Asymmetrical leg weakness, sensory level and
early bladder involvement.
• Recovery usually begins after a week of onset.
Transverse Myelitis

64. Physical Examination
• Tenderness over the spine may point to trauma
or infection.
• Increased tone, spastic weakness, legs more
than arms
• Reflexes are usually brisk, with positive
Babinski sign.
• Sensory ataxia, a sensory level.
• Sphincter dysfunction.
Transverse Myelitis

65. Associated Conditions
• Connective tissue diseases, e.g. SLE, JRA,
sarcoidosis, vasculitis…
• Rarely seen in association with metabolic
causes of myelopathy such as vitamin B12
deficiency.
Transverse Myelitis

66. DIAGNOSTIC CRITERIA FOR
TRANSVERSE MYELITIS
• Bilateral (not necessarily symmetric) sensorimotor and
autonomic spinal cord dysfunction
• Clearly defined sensory level.
• Progression to nadir of clinical deficits between 4 hours
and 21 days after symptom onset.
• Demonstration of spinal cord inflammation:
cerebrospinal fluid pleocytosis or elevated IgG index
or MRI revealing a gadolinium-enhancing cord lesion.
• Exclusion of compressive, postradiation, neoplastic,
and vascular causes.
Transverse Myelitis

67. Treatment
• Symptomatic management of bowel and bladder
dysfunction.
• Management of respiratory, cardiovascular &
autonomic dysfunction.
• IV methylprednisolone .
• IV immunoglobulin (IVIG) or plasmapheresis.
• Cyclophosphamide has been reported to be
useful in myelitis associated with systemic
inflammatory diseases
• Physical and occupational therapy may help
promote functional recovery and prevent
contractures.
Transverse Myelitis

68. Prognosis
• 50% make a full recovery within 3 to 6
months.
• 40% recover incompletely.
• 10% don’t recover.
• Older age, increased deep tendon reflexes, and
presence of Babinski sign may indicate better
course.
• Rapid progression, back pain, and spinal shock
predict poor recovery.
Transverse Myelitis

69. THANK YOU