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SAVING THE NERVES
- A CASE REPORT
Dr.Navas Shareef
nasmbbs@gmail.com
DNB Family Medicine
• 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
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 ??????
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
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
SEVERE MOTOR-SENSORY AXONAL POLYNEUROPATHY OF
BOTH UPPER LIMB AND LOWER LIMB
NERVE CONDUCTION STUDY
CSF ANALYSIS
FLUID TOTAL COUNT- 3 CELLS/mm3
TOTAL PROTEIN- 240 MG/DL
GLUCOSE- 82 MG/DL
NEUTROPHILS- NIL
NO ORGANISMS SEEN
CONTRAST MRI LS SPINE
PATCHY NERVE
ROOT
ENHANCEMENT AT
THE LEVEL OF
CONUS & S1-S2
LEVEL
 Rapid progressive ascending paralysis.
 Preceding gastritis
 Nobladder involvement /No sensory level /
Nofever at onset
 MRI: Nerve root enhancement
 NCS: Motor sensory axonal neuropathy
DIAGNOSIS
GUILLAIN-BARRE SYNDROME ?
PROGNOSIS
GBS
HOW TO
INVESTIGATE AND
CONFIRM
WHICH
VARIANT
OF GBS
HOW TO TREAT
AND TREATMENT
BARRIERS
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.
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.
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
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%
≥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
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
AIDP AMSAN
GULLAIN BARRY SYNDROME-VARIANTS
AMANMFS
Bickerstaff
encephalitis ParaparesisPharyngeal-cervical-
brachial weakness
DEMYELINATING AXONAL
OTHERS
LABORATORY FEATURES: EMG and NCS
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
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.
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
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
• 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
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
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
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
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
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.
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

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GUILLAIN-BARRE SYNDROME

  • 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
  • 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