Guillain barre syndrom, transverse myelitis and polio myelitis, traumatic neuritis

1. Approach to a child
with
ACUTE FLACCID
PARALYSIS
Dr. Tasnima Nowrin(MD Phase
A)
Dr. Nusrat Ajmir(MD Phase A)
Dr. Musfika Jahan(DCH
student)

2. Case Scenerio
 Abir, 8 years old boy, hailing from
swandip, admitted in cmch with the
complains of
Difficulty in swallowing and talking with
for last 3 days.
Weakness of both upper limbs for last
5 days.
Weakness in both lower limb for 8
days.
Unable to walk for same duration.
H/O fever with cough 20 days back.

3. INTRODUCTION
 Acute flaccid paralysis (AFP) is a
clinical syndrome characterized by
rapid onset weakness, that many
times includes respiratory and bulbar
weakness.
 AFP is a broad clinical entity with an
array of diagnostic possibilities.
 An accurate and early diagnosis of the
cause has important bearing on the
management and prognosis.

4. DEFINITION
 Any child less then 15 years old with:
 Acute: Rapid progression from
weakness to paralysis (sudden onset)
 Flaccid: loss of muscle tone, floppy-
as opposed to spastic or rigid.
 Paralysis: Inability to move the
affected part (loss of voluntary
movement)
 And the paralysis is not from birth and
is not a result of an injury.

5. Diagnostic Approach
 Every child with AFP is a medical
emergency requiring systematic
evaluation and management.
 Initial assessment of any such acutely
ill child should concentrate on rapid
cardiopulmonary assessment and
resuscitation.

6.  Following are the key areas on initial
assessment;
 1. Detect and manage respiratory
muscle weakness.
 2. Detect and manage bulbar
weakness.
 3. Evaluate for cardiovascular instability.
 4. Rule out dyselectrolytemia or
toxemia.
 5. To rule out a spinal compression
(traumatic, intraspinal collections)

7.  The first step is to determine if an
unwell child actually has muscle
weakness.
 Pseudoparalysis due to limb pain may
result from trauma, arthritis/arthralgia,
myostis, joint or periosteal bleeds or
joint or periarticular infections or
inflammations.
 It is useful to remember the possible
causes of AFP in children using a
neuro-anatomical approach.

8. Differential Diagnosis of AFP

9. Guillain Barre Syndrome
(GBS)
 Post-infectious
 Acute, rapidly progressing
 Ascending
 Potentially fatal form of
polyneuritis
 Involving mainly motor but
sometimes also sensory and
autonomic nerves
 Also known as: Acute
inflammatory demyelinating
polyneuropathy .

10. Epidemiology:
 0.1-2.4 cases in per lac population.
 0.38-0.91 incidence in childern.
 Occurs rarely in children <2 years.
 Males > Females
Etiology:
 Follows infection by 10 days.
 Occurs after an infection triggered immune
mediated attack on the nerve axons or
myelin.
 Antecedent respiratory or gastrointestinal
illnesses are commonly found in the history.

11.  Gastrointestinal tract
infection:
(AGE, 20% 2-4 week
back)
 Campylobacter jejuni
 Helicobacter pylori
 Respiratory infection:
(2/3rd cases, 40% 1
month before onset)
 Mycoplasma
pneumoniae
 Zika virus
 Vaccine:
 Rabies vaccine
 Influenza vaccine
 Conjugated
meningococcal vaccine
 Others:
 Mononucleosis
 Lyme disease
 CMV

12. Pathogenesis
Molecular
mimicry
 Cross-reactive
immune attack by
host Antibody & T
cell which are
directed against
the pathogen &
nerve
components
(myelin to cause
demyelization).

13. Clinical Features
 Weakness
 Onset is gradual and progresses over
weeks
 Lower extremities (unable/refusal to
walk) > trunks >upper limbs > bulbar
muscles (50%)(laudry ascending
paralysis)>Facial nerve /cranial nerve
involvement (45%)> Autonomic
involvement(12-25%)
 Initial phase(2-4week)>platue
phase(days to 4 week)>recovery(6

14.  Proximal and distal muscles are
involved relatively symmetrically, but
asymmetry is found in 9% of patient
 Muscle tenderness /pain– At the
onset(67%)
 Maximum severety of weakness by 4
weeks
 Areflexia (83%), 10% retain reflex
throughout

15. Bulbar involvement (50%)
 Dysphagia and facial weakness – signs of
impending
 respiratory failure
Cranial nerve involvement (45%)
 o Facial nerve , Oculomotor nerve, ix, x,
xi, xii
Autonomic involvement
 Lability of blood pressure
 Profound bradycardia
 Occasional asystole
 Urinary retention or incontinence (20% of
cases)

16. Classification
ACUTE:
1. Acute inflammatory demyellinating
polyneuropathy(AIDP):
 Mainly caused by CMV
 Autonomic involvement
 Cranial nerve involvement is more
 Mainly occurs in western country

17. 2. Acute motor axonal neuropathy:
 Common in developing country
 Mainly caused by C. jejuni (75%)
 Less autonomic involvement
3. Acute motor sensory axonal
neuropathy(AMSAN):
 Main organism C. jejuni
 Recovery poor

18. 4. Miller-Fisher syndrom:
 Ataxia
 Areflexia
 Acute external ophthalmoplegia
Chronic inflammatory demyelinating
polyradiculoneuropathy:
 Slow progression (months to years)
 Recur intermittently(relapsing)
 Cranial nerve and autonimic
involvement uncommon

19. Investigations
CSF study:
 Characteristic albumino cytological
dissasociation evident after 7 days of
onset of symptoms.
 Elevation of CSF protein (more than
twice upper limit of normal)
 Cell content of CSF is normal (<10
cells/mm³)
 Glucose level normal
 Bacterial and viral culture is negative

20. Nerve conduction study:
 Motor nerve conduction velocities are
reduced
Electromyography:
 Evidence of acute denervation of
muscle

21. Treatment
 Intravenous immunoglobulin (IVIG):
400mg/kg/day for 5 consecutive days
or 1gm/kg/day for 2 days
 Plasmapheresis
 Steroids are not effective
 Neuropathic pain by narcotic
analgesic (gabapentine)
 Supportive management

22. Prognosis
 Spontaneous recovery begins within
2–3 weeks.
 Most regain normal muscular
function.(>90%)
 Improvement usually follows a
gradient inverse to the direction of
involvement.
 Mortality is low(1-2%) and gradually
results from respiratory failure, cardiac
arrythmia, hemodynamic instability

23. Transverse Myelitis
• The term transverse means across the
width, myelitis refers to inflammation of the
spinal cord. So transverse myelitis means
inflammation across the width of spinal
cord .
• The characteristic features of TM is rapid
development of both motor and sensory
deficits at any level of spinal cord. It
presents acutely as either partial or
complete cord involvement with bilateral
signs with a clear sensory level.

24. ETIOLOGY:
1. Idiopathic
2. Secondary:
 e.g. Immune mediated (post-
infectious or antibody driven)
3. Direct infection (infectious myelitis)

25. Two forms:
 Age 5yrs and younger –
 Develop spinal cord dysfunction over
hours to a few days
 Clinical loss of function Severe and
complete
 Recovery slow and incomplete
 Older children
Onset-Rapid
Recovery-Also rapid and complete

26. Clinical features
 TM is often preceed within the
previous 1-3 week by a mild
nonspecific illness, minimal trauma or
perhaps an immunization.
 Discomfort or overt pain in the neck
or back, depending on the level of
lesion is common.
 Depening on the severity, the
condition progress to numbness,
anesthesia, ataxia, areflexia and
motor weakness.

27.  Paralysis begins as flaccidity
(paraperesis/ tetraperesis) but over a
few weeks spasticity develops
 Urinary retention is a common and
early finding, incontinence occur later
in course
 Other findings may include priapism
or respiratory compromise, as well as
spinal shock.

28. Diagnostic evaluation
 Acute TM is a diagnosis of exclusion, a
thorough evaluation should be
completed in all cases
 MRI of spine:
Gadolinium enhanced MRI of spine
shows-Mild fusiform swelling of cord over
several segment, most frequently in
thoracic segments (hyperintensity in T2
image)
• CSF study:- Cytology: Moderate
pleocytosis (>10 lymphocytes/mm³)
-Level of CSF protien maybe elevated or
normal
- Ig G index : elevation of IgG index

29. Treatment
 There are no standards for the treatment
of TM
 Available evidence suggests modulation
of immune response may be effective in
decreasing severity and duration of
disease
 Initial approach is high dose steroid
particulary methylprednisolone
 If there is poor response other
approaches are :
Intravenous immunoglobulin
Plasma exchange

30.  If TM is secondary to underlying
antibody driven disorder, treatment
such as rituximab or
cyclophosphamide can be considered
 Long term prophylactic therapy is
recommended for children with
recurrent forms of the disease

31. Poliomyelitis
 Polio= grey (greek word)
 Myelitis= inflammation of the spinal
cord
 First described by Michael
Underwood in 1789
 First Medical report on
poliomyelitis was described in
1840
 Most affects children < the age of
5 years in developing tropical
countries.
 IPV developed by Dr. Jonas Salk
and first used in 1955
 OPV developed by Dr. Albert
Sabin and first used in 1961
 Bangladesh declared polio free in

32. Poliomyelitis
 It is a viral infection Enterovirus (RNA)
 Rapidly inactivated by heat,
formaldehyde chlorine, ultraviolet light
 It is contagious: usually spread from
person to person.
 Incubation period ranges from 6 to 20
days
 Only harmful to humans
 Virus localized in the anterior horn cells
of the spinal cord and certain brain
steam motor nuclei

34. Poliomyelitis
Pathogenesis
Entry into mouth
Replication in GI tract,
local lymphatics
Hematologic spread to
lymphatics and CNS
Viral spread along nerve fibers
Destruction of motor
neurons (AHCs)
Destruction of the spinal cord
occurs focally and within 3 -
6days .

35. Clinical Presentation
 Acute stage: generally lasts 7 to 10 days.
 fever
 pharyngitis
 headache
 anorexia
 nausea
 vomiting.
 Illness may progress to aseptic
meningitis.
 Symptoms range from mild malaise to
generalized encephalomyelitis with
widespread paralysis.

36. Clinical course
 Paralytic disease occurs 0.1% to 1% of
those who become infected with the
polio virus.
 Hyperesthesia or paresthesia in
the extremities and muscular
pain is common
 Paralysis of the respiratory muscles or
from cardiac arrest if the neurons in the
medulla oblongata are destroyed.
Paralysis occurs twice as often in the
lower extremity as in upper extremity.

37. Diagnosis
 Characteristic clinical manifestation.
 Isolation of virus from stool is
comfirmatory.
 2 sample of 8-10gm stool each to be
collected 24 hours apart within 14 days
of onset of AFP and to be sent in cold
box at 4 to 8 degree centigrade.
 PCR is the method of choice for polio
detection.
 CSF study: color-clear, cell- 20-
300/mm3. protein normal

38. Treatment in the acute stage
Bed rest, analgesics, hot packs, and
anatomical positioning of the limbs
Close monitoring of respiratory and
cardiovascular functioning is essential
during the acute stage of poliomyelitis
along with fever control and pain relievers
for muscle spasms.
Mechanical ventilation, respiratory
therapy may be needed depending of the
severity of patients.

39. Physiotherapy
Gentle passive exercises of all joints
Physical therapy is recommended
for full recovery.
Passive stretching exercises for mild to
moderate contractures
Surgical release of tight fascia and
lengthening of tendons may be necessary
for contractures persisting longer than 6
months.
Orthoses should be used until no
further recovery is anticipated.

40. Prognosis
Patients have some or
full recovery from
paralysis, most clinical
recovery occurs during
the 1 month and
almost complete
within 6 months.
Limited recovery may
occur for about 2
years.

41. Traumatic Neuritis (Following
Injection)
 Traumatic neuritis is suspected in
cases in which there is one limb
involvement and definite history of
injection in that limb (usually less
than 24 h) before the onset of
paralysis.
 It is associated with pain and
hypotonia of affected limbs.
 sensory deficits and lack of CSF
pleocytosis favor the diagnosis of
traumatic neuritis.

42. Acute spinal cord compression
• Trauma to the back
• Spinal epidural abscess
• Vascular anomalies of the cord
• Spinal cord tumors.
 Clinically difficult to differentiate
from Transverse Mylitis
 CT scan of the spine or MRI are sensitive
and can show the nature of obstruction

43. Hypokalemic Paralysis
 This is an important differential in any
child particularly in a younger child with
AFP.
 An early recognition can prevent
potentially fatal cardiac complications.
 In the developing countries, it most
commonly results from diarrheal
diseases.
 Rarer familial chanellopathies,
underlying disorders, such as renal
tubular acidosis, primary/secondary
hyperaldosteronism also need to be
considered.
 Correction of potassium levels rapidly

44. Differentiating among common
causes of AFP

45. Management of a case of
AFP
1. ABCs
 Ensure protection of airway and
adequate ventilation (especially if there
is respiratory muscle weakness, shallow
respiration, dysphagia, weak gag)
 Check and support: BP and Heart Rate
 Immobilize neck if history of neck/head
trauma
 Send electrolytes and get an ECG- to
look for hypokalemia
2. Examination and classification into
pattern

46. 3. Investigations (according to the
suspected site of lesion and cause of
paralysis)
 Neuroimaging (spinal cord)
 MRI indicated in all cases of
myelopathy, suspected transverse
myelitis
 Electrophysiologic testing (NCV &
electromyography): Guillain Barre
syndrome
 Lumbar puncture (CSF): Guillain Barre
syndrome
 Biochemistry: Creatine Kinase,

47. 4. Treatment (depends on the
underlying etiology identified)
 All children: meticulous supportive
care, anticipate and identify
respiratory, bulbar weakness shock
due to reduced vascular tone (spinal
cord disease), Autonomic instability,
complications of immobilization and
prevention of nosocomial infections.
 Specific therapy: according to
underlying etiology.

48. Examination findings of Abir
 Patient was ill looking, conscious, co-
operative
 Bi lateral incomplete closure of both eye
lid was present
 Bp- 130/90 mmHg
 Cranial nerve examination: bilateral
lower motor type facial nerve palsy
 Motor function of upper and lower limb:
◦ muscle power 2/5 in all 4 limbs
◦ Tendon reflexes absent
◦ Bilateral planter reflex absent
 Senrory function: intact

50. How did we treat Abir
 Abir was treated in pediatric ICU of
CMCH with
I. Parenteral councelling
II. Oxygen inhalation
III. NG tube feeding
IV. Broad spectrum antiobiotic due to
aspiration pneumonia
V. Antihypertensive due to autonomic
involvement

51. • IVIG was given in 2 consecutive
days as 1gm/kg/day dose regimen
• Total 38 vial IVIG was given in one
day (wt 19kg, 500mg/10cc per vial)
• Dramatic improvement was noted
after administration of IVIG.
• Physiotherapy was given during
recovery period.