A condition in which the immune system attacks the nerves. The condition may be triggered by an acute bacterial or viral infection. Symptoms start as weakness and tingling in the feet and legs that spread to the upper body. Paralysis can occur. Special blood treatments (plasma exchange and immunoglobulin therapy) can relieve symptoms. Physiotherapy is required.

1. CASE
PRESENTATION
On Guillain–Barré syndromeAcute motor axonal
neuropathy (AMAN) variant

2. DEMOGRAPHIC PROFILE:
Name: Aadil khan
IP number: 014642
Age: 8 years
Sex: Male
Ward: 3rd floor
Bed No: 304
Date of birth: 6th august 2012
Date of admission: 21/09/2020
Diagnosis: Guillain–Barré syndrome (AMAN) Acute motor axonal neuropathy
variant with fungal meningitis with B/L pneumonia
Educational status of mother: Educated
Father name: Mr.abdullah khan
Address: Araria district ,bihar

3. HISTORY OF THE CHILD:
Chief complaint:
 Abnormal jerky movement
Present medical history:
According to mother patient had 1 episode of fever and
after that abnormal movements like unrolling of eyeballs,
deviation of mouth to one side, tonic clonic movements of
upper limbs started. After that the patient become
unconscious and was rushed to Holy family hospital
emergency. In emergency he was sedated and a fever of 40
degree was recorded, from emergency patient was admitted
into PICU

4. Past medical history:
There was no history of similar episodes in past.
K/C/O GBS(AMAN) on CPAP
Disease onset was in July 2017,took treatment from CNS Patna
with complaint of pain and weakness in both lower and upper
limbs and fever for 4 days where the child was diagnosed with
GBS . tracheostomy done on 25th July 2017.
Present surgical history:
No surgical history
Past surgical history:
No significant of past surgical history.

5. Birth history; -
Antenatal history: -
 mother did not have any major ailment or any infection or any exposure to the
radiations during her antenatal period
 had visited nearby hospital 4 times for her routine check-up
 taken two doses of T.T vaccines
 took iron, calcium and folic acid tablets regularly
Intra natal history; -
 Had normal vaginal delivery at government hospital
 child injury; no
 Childs birth weight was 2.5kg
Neonatal history; -
 Colour of the baby at the time of birth was pink
 Cried immediately after birth
 No eye discharge/infection was there
 Passed meconium and urine within first 24 hour of life

6. FAMILY HISTORY:
Type of family: - nuclear
Family medical history; - there is no family
history of any chronic illness in the medical history
of the family.

7. PERSONAL HISTORY;
 Child is non-vegetarian
 Sleeps at least 10-12 hours a day
 Child has passed urine and stool
SOCIOECONOMIC HISTORY:
Type of house: concrete
Ventilation: adequate
Water supply: tap water
Drainage system: covered
Toilet facility: own latrine
Medical facility: generally, go primary health centre for minor ailments
Religion; Muslim
Occupation of parents: private job
Total income of the parents: 15,000-20,000month

8. IMMUNIZATION HISTORY
Immunization of the child is complete
a) Physiological measurement
IN CHILD NORMAL
Temperature 40’C 36.5-37.5’C
Pulse 100b/m 80-90b/m
Respiration 26b/m 20-30b/m
Blood pressure 127/79(88)mm/hg 120/60mm/hg

9. General appearance:
 Consciousness: conscious
 Activity: alert
 Cleanliness: hygiene maintained
 Body built: slightly over weight
 Nourishment: child is slightly over weight
SKIN
 Colour: pallor
 Temperature: 40 degree Celsius.
 Nails : no clubbing was present
HEAD & SCALP:
 Size: normal
 hair: normal black hair

10. EYES:
 Eye brows: symmetric and evenly distributed
 Eye lids: normal
 Eye lash: normal distribution and black in colour
 Sclera: normal
 Conjunctiva: normal whitish in colour
 Eyeball: normal
 Pupil: reactive to light
EARS:
 Hearing ability: normal
 External canal: no any abnormality
 Discharges: no discharge found
NOSE:
 Nasal septum : centrally located, no deviation
 Rhinorrhoea : not present
 Discharges: no discharge
 Nasal polyp: no any polyp is present

11. MOUTH & THROAT:
 Lips: dry
 Breath: normal
 Tongue: dry and pink
 Teeth :
 Gums: healthy
 Throat: no swelling
NECK:
 Thyroid gland: normal
 Lymph node: not palpable
CHEST:
 Shape: symmetric
 Respiratory rate: 26 breaths/min
 Respiratory sound: crackles audible B/L (L>R), Air entry equal in both
lungs
 Heart rate: 100b/min
 Heart sound: S1 and S2 heard

12. ABDOMEN:
 Inspection:
 abdomen is flat and symmetry is normal
 scar or lesion is not present
 Palpation:
 abdomen of the child is soft and tenderness is not present
 no mass was palpable
 Auscultation: bowel sound normal
 Percussion:
 absent of gas and fluid
 no sign of ascites and peritonitis seen
BACK AND SPINE:
 Posture: normal
GENETALIA:
 Lymph nodes: no lymphadenopathy found
 Urethral opening: normal
 Testes: no abnormalities found
 Congenital defects: not any

13. ANUS:
 Sphincter control: child is passing
stool in diaper
 Lesions: absent
 Inflammation: absent
EXTREMITIES:
 Gait: cannot be observed
 Mortality: immobile
Deformities: none

14. GUILLAIN-BARRÉ SYNDROME
A collection of clinical syndromes that manifests as
an acute inflammatory
polyradiculoneuropathy(Polyradiculopathy refers
to damage to multiple nerve roots sufficient to
produce neurologic symptoms and signs such as
pain, weakness, and sensory loss. ) with resultant
weakness and diminished reflexes.

15. The classic presentation is characterized
by an acute monophasic(single phase),
non-febrile, post- infectious illness
manifesting as ascending weakness and
areflexia
Sensory, autonomic, and brainstem
abnormalities may also be seen.
With the eradication of poliomyelitis,
GBS is the most common cause of
acute motor paralysis in children.

16. PATHOGENESIS
The pathogenesis of GBS remains unclear

17. Increasing data indicate that it is an autoimmune disease,
often triggered by a preceding viral or bacterial infection
with organisms such as:
Campylobacter jejuni
Cytomegalovirus
Epstein-Barr virus
Mycoplasma pneumoniae.
Vaccination against the:
Flu
Rabies
Meningitis
Are documented precipitating factors

18. PATHOPHYSIOLOGY
Two pathophysiological forms have been
described:
Demyelinating form of GBS :- Segmental
demyelination of peripheral nerves is due to
immune mediated involving both humoral
and cell- mediated immune mechanisms
Axonal forms of GBS :- axonal degeneration
may occur without demyelination or
inflammation
2/3 of patients have a history of an
antecedent gastrointestinal or respiratory
tract infection

21. The mechanism of disease possibly involves an abnormal T-cell
response precipitated by an infection which activate CD4+ helper-
inducer T cells

22. EPIDEMIOLOGY
The annual incidence of GBS range from 0.5-1.5
cases per 100,000 population in individuals
younger than 18 years
No evidence exists for any racial predilection
Males appear to be at greater risk for GBS than
females

24. TYPES OF GBS
-ACUTE INFLAMMATORY DEMYELINATING
POLYRADICULONEUROPATHY(AIDP)
-ACUTE MOTOR AXONAL NEUROPATHY(AMAN)
-ACUTE MOTOR AND SENSORY AXONAL
NEUROPATHY(AMSAN)
-MILLER-FISHER SYNDROME(MFS)
-POLYNEURITIS CRANIALIS

25. ACUTE INFLAMMATORY DEMYELINATING
POLYRADICULONEUROPATHY(AIDP)

26. ACUTE MOTOR AXONAL NEUROPATHY(AMAN)

27. ACUTE MOTOR AND SENSORY AXONAL
NEUROPATHY(AMSAN)
 This type is rare and resembles
AMAN except sensory nerves are
also affected.
 This type is associated with a
severe course and poor prognosis.

28. MILLER-FISHER SYNDROME(MFS)

29. POLYNEURITIS CRANIALIS
This is an acute onset of Multiple CN
palsies (usually bilateral CN VII with sparing
of CNs I and II)
Elevated cerebrospinal fluid protein
Slowed nerve conduction velocity
 Uncomplicated recovery.

30. Physical Examination
• An ascending motor weakness is noted along with
areflexia in the classic form.
• Areflexia is a hallmark of GBS.
• Occasionally, some of the more proximal reflexes
still may be elicited during the early phase of the
disease.
• Progression from normoreflexia / hyporeflexia to
areflexia is consistent with acute features of GBS.

31. Occasionally:
Autonomic instability (26%)
Ataxia (23%)
Dysesthesias (20%)
Cranial nerve findings (35-50%),
predominantly facial palsy (Children>adult) are noted.
• Leg weakness (i.e., foot drop) is usually noticed first and
weakness eventually involves the calves and thighs.
• Later, respiratory muscles and upper extremities show
involvement.

32. • Some children may become non-ambulatory.
• Weakness also may involve the respiratory muscles,
and some children need respiratory support during the
course of the disease.
• Mechanical ventilation is used until respiratory muscle
function returns.
• The autonomic neuropathy involves both the
sympathetic and parasympathetic systems;
manifestations include: Orthostatic hypotension,
Hypertension ,Pupillary dysfunction ,Sweating
abnormalities ,Sinus tachycardia

33. Diagnosis
The diagnosis of GBS is typically based on the
presence of : o Progressive ascending
weakness o Areflexia
Lumbar puncture
Electrodiagnostic studies
MRI (occasionally) Can give support for the
diagnosis.
• Abnormalities on these studies do not develop
until days to weeks after onset of symptoms.

34. Treatment
• In pediatrics, the most effective form of therapy is
generally considered to be intravenous
immunoglobulin (IVIG)
• Plasmapheresis may also be used

35. Prognosis
• In general, the outcome of GBS is more favourable in
children than in adults • the recovery period is long, often
weeks to months
• Rarely, it can be fatal in 5-10% of patients with respiratory
failure and cardiac arrhythmia
• Recurrence of GBS occurs in approximately 5% of cases
• Overall mortality rate in childhood GBS is estimated to be
less than 5%
• Deaths are usually caused by respiratory failure, often in
association with :
Cardiac arrhythmias
Dysautonomia