Guillain barry syndrome - a case report.

1. SAVING THE NERVES
- A CASE REPORT
Dr.Navas Shareef
nasmbbs@gmail.com
DNB Family Medicine

2. • Conscious, oriented to
time, place and person.
• Pulse: 80/min, RR-
20/min
• BP:110/70 mm Hg
• GCS 15/15
• No PICCLE
1. Epigastric pain for 3
days
2. Vomiting 2 episodes
3. Constipation for 3 days
4. Tiredness
Cardiovascular
system:S1,S2 normal
Respiratory system: Bilateral
NVBS
Per abdomen: Epigastric
tenderness +, BS +
Neurological examination-
Grossly normal
CASE
DESCRIPTION
• 65 year old male
• Farmer
• No known
comorbidities

3. FLUID TOTAL COUNT- 3 CELLS/mm3
GLUCOSE- 82 MG/DL
NEUTROPHILS- NIL
NO ORGANISMS SEEN
PROGRESSION
DAY 01
DAY 02
8AM
DAY 02
2PM
DAY 04
Admitted in ward as acute gastritis and
started Rx symptomatically
C/o severe pain neck, back, shoulder and
limbs. Tingling and dysasthesias in extremities.
Worsening of weakness of limbs
Emergency NCS and MRI spine done- shifted
to ICU………..
Intubated and started mechanical ventilation
DAY 30 ??????

4. HMF - Normal
Cranial nerves- within normal limits.
Motor System- Right Left
Bulk Normal Normal
Tone UL Flaccid Flaccid
LL Flaccid Flaccid
Power UL Proximal 5/5 5/5
Distal 3/5 3/5
LL Proximal 3/5 3/5
Distal 2/5 2/5
Plantars Flexor Flexor
DTR Biceps Brachioradialis Triceps Knee Ankle
Right - - - - -
Left - - - - -
Cerebellar system- within normal limits
Sensory system- Decreased sensation to touch, vibration in bilateral lower limbs.
Single breath count- 15 and showed progressive worsening

5. POLYNEUROPATHIES
 A/c intermittent porphyria
 GBS
 Tick paralysis
 Thallium poisoning
ACUTE MYELOPATHIES
 Spinal cord compressing
lesion
 Anterior spinal artery
syndrome
 Acute transverse myelitis
MOTOR NEURON DISORDERS
 Amyotrophic lateral sclerosis
NEUROMUSCULAR
JUNCTION DISORDERS
 Botulism
 Myasthenia gravis
 Lambert-Eaton
syndrome
DIFFERENTIAL DIAGNOSIS OF FLACCID PARAYSIS

6. SEVERE MOTOR-SENSORY AXONAL POLYNEUROPATHY OF
BOTH UPPER LIMB AND LOWER LIMB
NERVE CONDUCTION STUDY

7. CSF ANALYSIS
FLUID TOTAL COUNT- 3 CELLS/mm3
TOTAL PROTEIN- 240 MG/DL
GLUCOSE- 82 MG/DL
NEUTROPHILS- NIL
NO ORGANISMS SEEN

8. CONTRAST MRI LS SPINE
PATCHY NERVE
ROOT
ENHANCEMENT AT
THE LEVEL OF
CONUS & S1-S2
LEVEL

9.  Rapid progressive ascending paralysis.
 Preceding gastritis
 Nobladder involvement /No sensory level /
Nofever at onset
 MRI: Nerve root enhancement
 NCS: Motor sensory axonal neuropathy
DIAGNOSIS

10. GUILLAIN-BARRE SYNDROME ?
PROGNOSIS
GBS
HOW TO
INVESTIGATE AND
CONFIRM
WHICH
VARIANT
OF GBS
HOW TO TREAT
AND TREATMENT
BARRIERS

11. FLUID TOTAL COUNT- 3 CELLS/mm3
GUILLAIN BARRE SYNDROME
GLUCOSE- 82 MG/DL
NEUTROPHILS- NIL
NO ORGANISMS SEEN
• Acute auto immune mediated polyradiculoneuropathy
• Overall world-wide incidence of 1 to 2 cases per 100,000
per year
• Most common preceding infection -Campylobacter jejuni
• Other agents –CMV, EBV, HIV and Zika virus
• Rarely after triggering event such as immunization, surgery,
delivery, trauma, and bone-marrow transplantation.

12. FLUID TOTAL COUNT- 3 CELLS/mm3
GUILLAIN BARRE SYNDROME
GLUCOSE- 82 MG/DL
NEUTROPHILS- NIL
NO ORGANISMS SEEN
Molecular mimicry :All GBSresults from
immune responses to non self
antigens(infectious agents, vaccines) that
misdirect to host nerve tissue through a
resemblance of epitope
Neural targets are gangliosides.

13. IMMUNOPATHOGENSIS
Conduction
block-
FLACCID
PARALYSIS
Disrupts
cluster of sodium channels
Complement
mediated injury at
Paranodal axon –
glial junction
Anti GB1 ab
as part of
molecular
mimicry
GM1 on
nerve,
nodes of
Ranvier

14. FLUID TOTAL COUNT- 3 CELLS/mm3
CLINICAL FEATURES
GLUCOSE- 82 MG/DL
NEUTROPHILS- NIL
NO ORGANISMS SEEN
• Progressive, fairly symmetric muscle weakness usually
starts in the legs
• Decreased or absent reflexes in affected arms or legs in
approx. 90% of patients at presentation and in all patients
as the disease progresses.
• Paresthesias in the hands and feet in >80% patients
• Pain in the back and extremities in 66% of patients
• Respiratory muscle weakness necessitating ventilator
support develops in 10 to 30%

15. ≥50%
Facial nerve palsies
50%
Oropharyngeal weakness
15%
Oculomotor weakness
70%
Autonomic dysfunction
 Wide fluctuation in BP:
Paroxysmal HTN &
orthostatic hypotension
 Diarrhea/Constipation
 Urinary retention
 Cardiac dysrhythmias
(mc- sinus tachycardia)
 Horner syndrome
 SIADH-Hyponatremia
CLINICAL FEATURES

16. UNUSUAL CLINICAL FEATURES
FLUID TOTAL COUNT- 3 CELLS/mm3
GLUCOSE- 82 MG/DL
NEUTROPHILS- NIL
NO ORGANISMS SEEN
• Papilledema
• Hearing loss
• Meningeal signs
• Vocal cord paralysis
• Mental status changes
• Reversible posterior leukoencephalopathy syndrome

17. AIDP AMSAN
GULLAIN BARRY SYNDROME-VARIANTS
AMANMFS
Bickerstaff
encephalitis ParaparesisPharyngeal-cervical-
brachial weakness
DEMYELINATING AXONAL
OTHERS

18. LABORATORY FEATURES: EMG and NCS

19. LABORATORY FEATURES:
LUMBAR PUNCTURE - CSF ANALYSIS
FLUID TOTAL COUNT- 3 CELLS/mm3
GLUCOSE- 82 MG/DL
NEUTROPHILS- NIL
NO ORGANISMS SEEN
Raised protein 45 to 200 or more mg/dL (0.45 to 2.0 g/L)
No pleocytosis. ie, <5 white blood cells/mm3
Albuminocytologic dissociation
Usually normal in <48 hrs.
Increased proteins at the end of firstweek
Think of HIV,CMV,Lymediseasein case ofpleocytosis.
Inflammation of nerve roots Increased permeability of
the blood-nerve-barrier CSF protein

20. SPINAL CONTRAST MRI
FLUID TOTAL COUNT- 3 CELLS/mm3
GLUCOSE- 82 MG/DL
NEUTROPHILS- NIL
NO ORGANISMS SEEN
• Thickening and enhancement of the intrathecal
spinal nerve roots and cauda equina.
• MRI brain: Enhancement of the oculomotor,
abducens, and facial nerves may be seen.

21. FEATURES MAKES THE DIAGNOSIS OF GBS DOUBTFUL
FLUID TOTAL COUNT- 3 CELLS/mm3
GLUCOSE- 82 MG/DL
NEUTROPHILS- NIL
NO ORGANISMS SEEN
• Sensory level (decrement or loss of sensation below a
spinal cord root level)
• Asymmetry of weakness
• Bowel and bladder dysfunction at onset
• Severe pulmonary dysfunction with little or no limb
weakness at onset
• Severe sensory signs with little or no weakness
• Fever at onset
• CSF pleocytosis with cell count >50/mm3

22. FEATURES MAKES THE DIAGNOSIS OF GBS DOUBTFUL
FLUID TOTAL COUNT- 3 CELLS/mm3
GLUCOSE- 82 MG/DL
NEUTROPHILS- NIL
NO ORGANISMS SEEN
• Sensory level (decrement or loss of sensation below a
spinal cord root level)
• Asymmetry of weakness
• Bowel and bladder dysfunction at onset
• Severe pulmonary dysfunction with little or no limb
weakness at onset
• Severe sensory signs with little or no weakness
• Fever at onset
• CSF pleocytosis with cell count >50/mm3

23. • ICU admission for close monitoring of respiratory,
cardiac and hemodynamic function.
• Ventilator support for neuromuscular respiratory
failure / Bulbar dysfunction (inability to clear secretions)
• Adequate pain control
• Prophylaxis for deep vein thrombosis
• Bladder and bowel care
• Physical and occupational therapy
• Psychological support
TREATMENT-SUPPORTIVE CARE

24. DISEASE-MODIFYING TREATMENT
IVIG vs PLASMAPHERESIS
• IVIG is not effective after 2 weeks of onset
• Plasma exchange can be done till 4 weeks of onset
• Both are equally effective
• Combination has no superior effect
• IVIG-thought as first choice since its easy to
administer
• Glucocorticoids are not beneficial

25. IVIG
 Rs. 15,849/- per 5gm 100ml
unit
 0.4gm per kg per day
 Average adult weighing 60kg
needs 5 unit per day for 5
days
 Total cost : 3,96,225/-
PLASMAPHERESIS
COST
 Rs. 512/- 450ML FFP UNIT
 50ml per kg per day
 Average 8 unit per day for
5 days
 Total cost : 20,480/-
AFFORDABILITY

26. PLASMAPHERESIS
8 UNIT FFP (50ml/kg) PER DAY FOR 5 CONESCUTIVE DAYS @ 40ML/MINUTE
1 LITRE NORMAL SALINE@ 40ML/MINUTE
HEPARIN 2000 IU PRIMING + 1000 IU/HOURS INFUSION
CALCIUM GLUCONATE 10% , 2 AMPOULE IN 50ML NS
DAILY 2 LITRE PLASMA EXCHANGE VIA DIALYSIS CATHETER FOR 5 DAYS
S/E – Hypotension, hypo prothrombinemia, bleeding, arrhythmia

27. IVIG
USE SEPARATE IV LINE
REFRIGERATED BOTTLE SHOULD BE BOUGHT TO ROOM TEMP
SLOW INUSION FOR THOSE WITH RENAL INJURY
START SLOWLY @ 25-50ML/HOUR AND INCREASE UPTO 100ML /HOUR
DAILY 0.4GM PER KG FOR 5 DAYS (Total:2000MG)
S/E – Anaphylaxis, Thrombophlebitis, Aseptic meningitis,
Hyper viscosity syndrome, Renal failure in dehydrated patients

28. FOLLOW UP
*Came to ER as a case
of GASTRITIS and later
diagnosed to have
GBS-AMSAN variant
ADMISSION POINT
TREATMENT PHASE
CONDITION AT DISCHARGE
FOLLOW UP-1 & 2 MONTH
*Mechanical ventilation
*PLASMAPHERESIS
*Intensive supportive care
*Vitally stable,
*Walk with support
*On Foley catheter
for urinary retention
*Patient was able to
ambulate sooner than
Predicted
*No residual muscle
weakness or autonomic
dysfunctions.

29. FLUID TOTAL COUNT- 3 CELLS/mm3
TAKE HOME MESSAGE
GLUCOSE- 82 MG/DL
NEUTROPHILS- NIL
NO ORGANISMS SEEN
• DTR examination for any type of weakness/ tiredness.
• Don’t hesitate to start immunotherapy in geriatric
group
• Mortality is determined by quality of ICU care
• Plasmapheresis is much cheaper compared to IVIG
• Do not use steroid
• Earlier the initiation of IVIG/PE, faster the recovery