Gillian barre syndrome presentation pptx

Ppt

1. Case presentation N.Sanjana Intern

2. Arun kumar , a 8 year old male child from varkatpally , yadadri Chief complaints- Weakness in both limbs since 7 days Pain in both lower limbs since 7 days

3. History of presenting illness--- He was apparently alright 7 days back then he developed weakness in both lower limbs, parents noticed stamping of foot in the morning 10 am, by afternoon he was unable to stand from sitting position. Next day he was unable to get up from lying down position. During the episode he was able to hold a glass of water and take feeds by himself, able to raise arms above the level of head. History of pain in both lower limbs since 7 days - started from the back of legs, gradually progressed to thigh, dragging type of pain.

4. No history of bowel, bladder disturbances, No history of altered sensorium, abnormal posturing of limbs, tightening of limbs, defective vision, abnormal sensation over body, facial deviation, dysphagia, vomiting. No history of trauma or recent loose stools, fever, respiratory illness. No history of rash over body, bite marks, recent vaccination.

5. Past history: not significant Family history : No history of similar illness in family. Birth history: Single/ term/ LSCS/birth wt of 2.75 kg/ cried immediately after birth/ No h/o NICU stay. Immunization history: Immunized as per schedule. Developmental history : Appropriate for age

6. Parameter Value Standard deviation Weight for age 14.8 kg -3.54 Height for age 110.2 cm -2.30 BMI 12.18 -3.09 General Examination : child is active and afebrile. Pallor is present. No icterus, cyanosis, clubbing or lymphadenopathy. Vitals at admission: Temp:97.8 deg F deg. F, PR:125/min, RR:30/min, BP- 98/84 mmHg Spo2:98% peripheries warm, peripheral pulsations present. Anthropometry at admission:

7. Systemic Examination : Central nervous system: HMF - conscious, oriented, speech normal Cranial nerves examination - within normal limits sensory: within normal limits Motor system: T one : both lower limbs - hypotonia power UL:3/5 LL:1/5 reflexes: absent Gait : not able to walk cerebellar signs - absent meningeal signs - absent

8. Per abdomen : Soft, non-tender. No hepatosplenomegaly. Bowel sounds regular. Respiratory system: B/L breath sounds heard, crepts with occasional wheeze , mild intercostal and subcostal retractions. Cardiovascular system :S1S2 heard normally, no murmur

9. A dmitted to paediatric ICU in view of acute flaccid paralysis, ?GBS. Monitored for further worsening of weakness and development of autonomic dysfunction . I nvestigations -- CBC, LFT, RFT, SE, Creatine kinase, URE were within normal limits. S tarted on IVIG at dose of (2 g/ Kg) and given over 5 days. Other supportive medications along with physiotherapy was given. Lumbar puncture was done and sent for CSF analysis, which showed increased CSF proteins with acellular counts( Albuminocytological dissociation). NCS shows features of Acute Motor Axonal neuropathy. On day 9 admission he had 2 episode of urine and bowel incontinence for which MRI spine was taken, which was showing normal study. Child improved gradually, Power of lower limb and upper limb increased, he was able walk without support, No other features of autonomic dysfunction present. lower limb power 4/5, upper limb – 5/5 ( after IVIG )

10. Differentials.. Poliomyelitis ( fever present, asymmetrical weakness) Myopathy Tick paralysis Snake bite ( neuroparalytic ) Initial presentation of neuromuscular junction disorder Acute intermittent porphyria Toxic neuropathies HIV infection

11. Guillain Barre syndrome is a rare disorder in which body's immune system attacks nerves and causes damage to the peripheral nerves. The nerve injury often causes muscle weakness, cause paralysis and sensitivity problems, including pain, tingling or numbness.

12. Demyelinating form of GBS destroys myelin sheath,preventing the nerves from transmitting signals to the brain.

13. TYPES: Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) The most common sign of AIDP is muscle weakness that starts in the lower part of your body and spreads upward. Miller Fisher Syndrome (MFS)  - ataxia, areflexia, opthalmoplegia Acute Motor Axonal Neuropathy (AMAN) Acute Motor-sensory Axonal Neuropathy (AMSAN)

14. ETIOLOGY Bacterial infection ( campylobacter , mycoplasma) Viral infection (zika virus) Surgeries Transplantation Anesthesia Preceding heat stroke Preceding vaccination ( influenza )

15. Pathophysiology

16. Autoantibodies : Anti GM1 and Anti GD1 antibodies Anti GQ1b antibody ( miller fischer variant)

17. Clinical features Acute flaccid paralysis Acute onset with decreased tone Ascending progression Symmetrical weakness Peaks by 2 weeks of onset Duration <4 weeks ( self limiting) Polyneuropathy Motor Sensory ( tingling, numbness, parasthesia , neuropathic pain ) Bilateral facial nerve involvement Autonomic changes( changes in blood pressure , heart rate)

18. Neuromuscular respiratory failure - demyelination of the nerves that innervate the diaphragm and inter costal muscles results. Unusual features Presence of fever Presence of bowel / bladder symptoms Involvement of EOM

19. Investigations Electrophysiological studies Nerve conduction velocity- decreased Single fibre electromyogram- evidence of denervation of muscle CSF analysis Albuminocytological dissociation ( discrepancy between cell count and protein) Increased levels of CSF protein but cell count of CSF is normal(<10 cells/cc)

20. Management Supportive care Ventiltory support IVIG- decrease progression of muscle weakness 2g/kg over 5 days Plasmapheresis (plasma exchange)– to remove abnormal antibodies,humoral mediators, complement proteins ( if not responding to IVIG)

21. Complications— Respiratory compromise Bulbar palsies Bed sores due to paralysis Paresthesias even after recovery

22. Sources https://www.ncbi.nlm.nih.gov/books/NBK532254/ Essential pediatrics - GHAI ( 10 th edition ) Nelson textbook of pediatrics (21 edition)

23. Thank you