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Clinical Case
Discussion
DR SHINDE VIRAJ ASHOK
JUNIOR RESIDENT 3
DEPARTMENT OF PHARMACOLOGY
Patient details
 Name - Shubhangi Sheshrao Deshbhartar
 Age / Sex – 22yrs / F
 Reg no – 1226958
 Diagnosis – Myasthenia gravis with
hypothyroidism
S/B AP ↓ ENT 2 on 23/07/2016
Chief complaints
 Dysphagia
Findings
 Oral cavity – WNL
 Indirect laryngoscopy -
WNL
 k/c/o myasthenia gravis
on medication since 1
year
Advise
 Physician opinion
S/B Physician ↓ 2 on 23/07/2016
Referred from ENT
Chief complaints
 k/c/o myasthenia gravis with
hypothyroidism since 2 year
 Diplopia since 1 ½ year
 Slurring speech since 1 ½
month
 Weakness of both upper &
lower limb since 1 ½ month
 Difficulty in deglutition with
regurgitation since 8 days
O/E
 P – 80 bpm
 BP – 100/60 mm of Hg
S/E
 RS & CVS – WNL
 CNS – Conscious Oriented
 Motor system
 Nutrition – average
 Power – 5/5
 Hand grip – 60%
 DTR 2+
 Plantar – b/l flexor
S/B Physician ↓ 6 on 23/07/2016
 No h/o ptosis
 Tab thyroxine 100µg
 Tab azaron ( azathioprine )50mg OD
 Tab omnacortil (Prednisolone)5 mg OD
Advice
Please admit patient in Female Medicine Ward
S/B Lecturer / AP on 23/07/2016 at
2 pm
 GC – Moderate
 Afebrile
 P- 78bpm
 RS – Clear
Vitals – Stable
 Advice
Plasmapheresis
Nephro call
 Same treatment was
continued on 24/07/2016
Treatment
 1 point DNS
 Inj neostigmine 0.5mg BD
 Inj atropine 0.6mg before
neostigmine
 Tab omnacortil (Prednisolone)
5mg OD
 Tab thyroxine 100µg OD
 ↑↑Tab azaron (azathioprine)
150mg OD
S/B Registrar/ SP ↓ med 6 on
25/07/2016
 Nasal twang - ++ ,Opthalmoplegia
 GC – moderate
 Afebrile
 P – 90bpm
 HS – normal
 RS – clear
 P/A – soft
Investigation
 Hb – 7.6gm/dL
 TLC – 4000 cells/ mm3
 Platelet – 298000/mm3
Treatment
 1 point DNS
 Inj neostigmine 0.5mg BD
 Inj atropine 0.6mg before neostigmine
 ↑↑ Tab omnacortil (prednisolone) 40mg
OD
 Tab thyroxine 100µg OD
 Tab azaron (azathioprine)150mg OD
 Tab distinon (pyridostigmine) 60mg BD
Diagnosis - Myasthenia gravis with
exacerbation
S/B Lecturer ↓ nephrology on
25/07/2016
 Kindly send patient for plasmapheresis
 Right sided double lumen HD catheter inserted
Advice
 HIV - negative
 HBsAg – negative
 B+ve
 Anti HCV
 Arrange 5 FFP
 Plasma filter
 Dialysis tubing
S/B Registrar ↓ med 6 on
26/07/2016
 GC – moderate
 Afebrile
 P – 78 bpm
 BP – 110/70mm of Hg
 RS – NAD
 HS – Normal
 CNS – NAD
Treatment
 1 point DNS
 Inj neostigmine 0.5mg BD
 Inj atropine 0.6mg before
neostigmine
 Tab omnacortil (prednisolone)
40mg OD
 Tab thyroxine 100µg OD
 Tab azaron (azathioprine) 150mg
OD
 Tab distinon (pyridostigmine) 60mg
BD
26/07/2016 at 11am –
Plasmapheresis - 1
 ↓ All Aseptic Precautions
double lumen Double Lumen
Catheter inserted ↓ local
anaesthesia procedure
uneventful
 Patient had hypotension during
Plasmapheresis
 1.5 litre plasma removed
 5 unit FFP + 1 litre NS infused
Advice
 Inj monocef (ceftriaxone) 1
gm iv BD
 Arrange 5 unit of Fresh
Frozen Plasma for next
session on 28/07/2016
 X ray chest PA view
S/B Reg ↓ med 6 / SP ↓ 6 on
27/07/2016
 GC – moderate
 Afebrile
 P – 78 bpm
 BP – 110/70mm of Hg
 RS – NAD
 HS – Normal
 CNS – NAD
Treatment
 1 point DNS
 ↑↑ Inj neostigmine 0.5mg TDS
 Inj atropine 0.6mg before
neostigmine
 Tab omnacortil 40mg OD
 Tab thyroxine 100µg OD
 Tab azaron 150mg OD
 Tab shelcal 500mg BD
 Tab distinon 60mg BD
S/B Lecturer ↓ Nephrology on
28/07/2016 - Plasmapheresis -2
Advice
 Plasmapheresis today
 2 litre volume exchange
 5 point FFP
 2 point NS
 1 point RL
 Inj calcium gluconate …post
plasma pheresis
 Repeat
 CBC
 PT/INR
 Serum electrolyte
S/B Lecturer ↓ Nephrology
28/07/2016 at 3.45 pm
 While shifting patient c/o
discomfort
 P – 150 bpm irregular
 SpO2 – 94%
 Inj Lasix 20mg iv stat
 Inj hydrocortisone 100mg iv
stat
 Drop in SpO2 to 70%
 Hence AMBU bag ventilation
done
 SpO2 – 98 % achieved
 Bipap connected SpO2 – 98%
maintained
 Semiconscious
 ?? Myasthenia Crisis
Advice
 Shift to Intensive Cardiac Care
Uunit & Continue bipap
 Urgent Ca++, CBC , PT/INR,
ABG
Transfer out notes to ICCU on
28/07/ 2016
 C/O myasthenia gravis with exacerbation
Treatment
 1 point DNS
 Inj neostigmine 0.5mg TDS
 Inj atropine 0.6mg before neostigmine
 Tab omnacortil (prednisolone) 40mg OD
 Tab thyroxine 100µg OD
 Tab azaron (azathioprine) 150mg OD
 Inj monocef 1 gm BD
 Tab shelcal 500mg OD
Receiving notes in ICU
28/07/2016
 P – 136bpm
 BP – 90/ 60 mm of Hg
 SpO2 – 100%
 Chest – clear
 Dyspnoeic
 Sr Ca++ - 10.2
 Sr Na+ - 142meq/L
 Sr K+ - 2.8 meq/ L
 Sr Mg++ - 3.8 meq /L
Treatment
 2 point NS fast
 Inj neostigmine 0.5mg TDS
 Inj atropine 0.6mg before neostigmine
 Inj monocef 1gm iv BD
 Tab omnacortil 40mg OD
 Tab thyroxine 100µg OD
 Tab azaron 150mg OD
 Tab shelcal 500mg BD
 Tab distinon 60mg BD
 Inj KCl 40meq in 1 point NS over 8hrs
S/B Reg ↓ med 6 on 29/07/2016
at 8 AM
 GC – moderate
 Afebrile
 P – 86 bpm
 BP – 100/70 mm of Hg
 U/O – 800ml
 RS – NAD
 HS – Normal
 CNS – NAD
 P /A – soft
Treatment
 Inj neostigmine 0.5mg TDS
 Inj atropine 0.6mg before neostigmine
 D5 Tab omnacortil 40mg OD
 Tab thyroxine 100µg OD
 Tab azaron 150mg OD
 Tab shelcal 500mg BD
 Tab distinon 60mg BD
 D3 Inj monocef 1gm iv BD
 pH – 7.4
 HCO3- - 22
 PCO2 - 25
S/B Senior Physician ↓ 6 on
29/07/2016 at 10.40AM
 P – 90bpm
 Opthalmoplegia ++
 No Cyanosis
 Chest – Clear
 Mild improvement in
deglutition
Treatment
 Inj neostigmine 0.5mg SOS
 ↑↑ Tab pyridostigmine 60mg
TDS
 W/H omnacortil
(prednisolone)
 D1/3 - Inj methyl
prednisolone 500mg OD
 Rest ct all
Intubation notes 29/07/2016
 ↓ All aseptic precautions patient was
intubated with ET tube size 7.5 mm AE
b/l equal fixed put on ventilatory
support SpO2 - 92%
 Procedure - uneventful
S/B Registrar ↓ ICU 29/07/2016 at
6PM
 GC – poor
 Unconcious
 On ventilatory support
 HS – normal
 Chest – clear
 SpO2 – 92%
 Sr T3 – 490 IU
 Sr T4 – 18 IU
 TSH – 0.01IU
Advice
 W/H Tab Thyroxine 100µg
 Rest ct all
S/B MO / Registrar /SP↓ ICCU at
30/07/2016 at 8 AM
 GC – moderate
 Afebrile
 P – 86 bpm
 Chest – clear
 BP – 110/70mm of Hg
 U/O – 650ml
 RS – NAD
 HS – Normal
 CNS – NAD
 P/A – soft non tender
Treatment
 D1 Inj levofloxacin 500mg OD
 D4 Inj monocef 1gm iv BD
 D2/3 Inj methyl prednisolone 500mg OD
 Tab azaron (azathioprine)150mg OD
 Inj neostigmine 0.5mg TDS
 Inj atropine 0.6mg before neostigmine
 Tab pyridostigmine 60mg OD
 Tab shelcal 500mg BD
S/B Lecturer nephrology at
30/07/2016
 c/o myasthenia gravis in
myasthenic crisis
 Intubated yesterday in
view of poor respiratory
efforts
 P – 80 bpm
 BP - 110/70 mm of Hg
 B/L AEBE
 Concious oriented / moves
limb on command
Advice
 Plasmapheresis today
 Arrange 5 @ FFP
 Send patient with accompanying
resident doctors by12.30PM
 High risk plasmapheresis
explained to relatives
 Sr Ca+ -9.8mg/dL
 Sr Mg+ -1.97mg/dL
 Sr Na+ -137mEq/L
 Sr K+ - 2.6mEq/L
S/B SP ↓ 6 at 30/07/2016 at 2 PM
 GC- not stable on ventilator
 Conscious afebrile
 P – 78 bpm
 B/L Clear
 HS – normal
Treatment
 ↑↑Tab pyridostigmine 60 mg QID
 Rest ct all
Investigation
 Sr urea -14mg/dL
 Sr creat – 0.7mg/dL
 T protein – 5.3gm%
 T Bilirubin – 0.7mg%
 ALP - 44 IU/L
 SGOT – 40 IU/L
 SGPT – 13 IU/L
S/B Lecturer ↓ Nephrology on
30/07/2016 - Plasmapheresis - 3
 c/o myasthenia gravis currently in
crisis on ventilatory support
plasmapheresis 3rd today
 No spontaneous breathing on
Intermittent Positive Pressure
Ventilation
Advice
 2 litre exchange
 5 FFP
 2 @ RL
 1 @ NS
 Inj calcium gluconate 10 cc post
plasmapheresis
 Post plasmapheresis – there was
spontaneous trigerring on
ventilator
Advice
 PT/INR , Sr electrolytes
 Tensilon (edrophonium challenge
test)
 Plan for IVIg
 Would offer plasamapheresis on
Monday with 5@ FFP &
accompanying medicine resident
@ 10 AM
S/B Registrar ↓ ICCU / SP ↓ 2 on
31/07/2016 at 8 AM
 Patient on ventilator
 GC – moderate
 Afebrile
 P – 82 bpm
 BP – 120/80 mm of Hg
 U/O – 1200ml
 RS – clear
 P/A – soft non tender
 CVS – S1S2
 CNS – conscious oriented
Treatment
 D2 inj levofloxacin 500mg OD
 D5 inj monocef 1gm BD
 D3/3 inj methylprednisolone 500mg OD
 Tab azoran 150mg OD
 Inj pyridostigmine 60 mg QID
 Inj neostigmine 0.5mg im SOS
 Inj atropine 0.6mg SOS before inj
neostigmine
 Tab shelcal 1 BD
 PT/INR - 13/1.11
S/B registrar ↓ ICCU / SP ↓ 6 on
1/08/2016 at 8 AM
 GC – not stable
 Afebrile
 P – 80 bpm
 BP – 130/90 mm of Hg
 U/O – 500ml
 RS – clear
 P/A – soft non tender
 CVS – S1S2
 CNS – NAD
 SpO2 – 100%
Treatment
 ↓↓Inj pyridostigmine 60 mg
TDS
 D3 inj levofloxacin 500mg OD
 D6 inj monocef 1gm BD
 Inj neostigmine 0.5mg im SOS
 Inj atropine 2cc BD
 Tab shelcal 1 BD
 Tab azoran 150mg OD
Transfer notes 11PM at
01/08/2016
 Here by transferring out this patient from ward 24 to
nephrology for plasmapheresis c/o myasthenia gravis with crisis
Treatment
 Inj pyridostigmine 60 mg TDS
 Inj neostigmine 0.5mg im SOS
 Inj atropine 0.6mg SOS before inj neostigmine
 D3 inj levofloxacin 500mg OD
 D6 inj monocef 1gm BD
 Tab azoran 150mg OD
S/B Lecturer ↓ Nephrology on
01/08/2016 Plasmapheresis - 4
 c/o myasthenia gravis with crisis
 BP – 120/ 80 mm of Hg
Advice
 Plasmapheresis today
 2 litre volume exchange
 5 point FFP
 2 point NS
 2 point RL
 Inj calcium gluconate …post plasma pheresis
S/B Registrar ↓ ICCU / SP ↓ 2 on
02/08/2016 at 8 AM
 GC – not stable
 Afebrile
 P – 86 bpm
 BP – 120/80 mm of Hg
 U/O – 2000ml
 SpO2 – 99%
 RS – clear
 CVS – S1S2
 CNS – conscious oriented
 Proximal muscle weakness +
 Opthalmoplegia +
Treatment
 Inj pyridostigmine 60 mg TDS
 Inj neostigmine 0.5mg im SOS
 Inj atropine 0.6mg SOS
before inj neostigmine
 Inj rantac 50mg BD
 D4 inj levofloxacin 500mg OD
 D7 inj monocef 1gm BD
 Tab shelcal 1 BD
 Tab azoran 150mg OD
S/B SP ↓ 6/ SP ICCU at
02/08/2016 at 10 AM
 GC – moderate
 Conscious
 Afebrile
 P – 80bpm
 Wt – 48 × 2 = 96gm
Advice
 Plasmapheresis
 Free T3 , T4 , TSH
 Endocrinologist call
 CVTS call
Treatment
 Ct all
 Inj IVIg 20gm/day × 5 days
-last day 15gm
03/08/2016
To CVTS surgeon,
Sir /madam ,
kindly evaluate this patient c/o hypothyroidism with
myasthenia gravis with thymoma patient is not improving on
plasmapheresis . Kindly advice regarding thymectomy
Thanking you
S/B by Dr Ashish
Advice
Thymectomy only after GC – improves
S/B registrar ↓ ICCU / SP ↓ 6 on
03/08/2016 at 8 AM
 Patient on ventilator
 GC – not stable
 Ventilatory support
 Afebrile
 P – 90 bpm
 BP – 110/70 mm of Hg
 RS – clear
 P/A – soft non tender
 CVS – S1S2
 CNS – conscious oriented
Treatment
 Inj pyridostigmine 60 mg TDS
 Inj neostigmine 0.5mg im SOS
 Inj atropine 0.6mg SOS before inj
neostigmine
 D4 inj levofloxacin 500mg OD
 D9 inj monocef 1gm BD
 Tab shelcal 1 BD
 Tab azoran 150mg OD
 Plasmapheresis
 Nephrologist reference
S/B Lecturer ↓ nephrology – 03/08/2016
Plasmapheresis – 5
 C/O myasthenia gravis with crisis received 4 plasmapheresis
Advice
Send patient accompanying medicine resident with 5 @
Fresh Frozen Plasma
 Plasma removal – 2 L
 Replace with 5 Fresh Frozen Plasma
 2 @ RL
S/B Registrar ↓ ICCU / SP ↓ 6 on
04/08/2016 at 8 AM
 GC – not stable
 Afebrile
 P – 82 bpm
 BP – 120/90 mm of Hg
 SpO2 – 100% on Ventilatory
support
 U/O – 1600ml
 RS – clear
 P/A – soft non tender
 CVS – S1S2
 CNS – conscious oriented
Treatment
 Inj pyridostigmine 60 mg TDS
 Inj neostigmine 0.5mg im SOS
 Inj atropine 0.6mg SOS
before inj neostigmine
 D5 inj levofloxacin 500mg OD
 D10 inj monocef 1gm BD
 Tab shelcal 1 BD
 Tab azoran 150mg OD
 Plasmapheresis
04/08/2016 Recovery Room
To
MO/ Registrar
Anaesthesia on call
kindly call over to evaluate this patient a c/o
myasthenia gravis on ventilation not maintaining
saturation on A/C mode and opine expertise management
Thanking you
S/B Anaesthetist on 04/08/2016
 C/O myasthenia gravis
 Conscious oriented
 Afebrile
 P – 83 bpm
 BP – 120/70 mm of Hg
 RS – clear
 CVS – S1S2
 SpO2- 96%
Ventilation
 Mode – NC/AC
 RR - 16 cycles/min
 Positive End Expiratory Pressure - 5
 TN – 360
 FiO2 -100%
Advice
 Propped up
 Ct ventilatory support
 Ct all
S/B Registrar ↓ ICCU / SP ↓ 6 on
04/08/2016 at 8 AM
 GC – not stable
 Afebrile
 P – 95 bpm
 BP – 130/70 mm of Hg
 SpO2 – 100% on Ventilatory
support
 U/O – 1300ml
 RS – clear
 P/A – soft non tender
 CVS – S1S2
 CNS – conscious oriented
Treatment
 Inj pyridostigmine 60 mg TDS
 Inj neostigmine 0.5mg im SOS
 Inj atropine 0.5cc before inj
neostigmine
 D6 inj levofloxacin 500mg OD
 D11 inj monocef 1gm BD
 Tab shelcal 1 BD
 Tab azoran 150mg OD
 Tracheostomy tommorrow
04/08/2016 at 1 AM
To ,
Medical officer / registrar
Anaesthesia on call,
kindly call over to evaluate this patient a c/o
myasthenia gravis on ventilation not maintaining
saturation on A/C mode and opine expertise management
thanks
04/08/2016 at 1.05 AM S/B
Anaesthesiologist Registrar
A case of myasthenia gravis
 Ventilation
 Mode VC/AC
 Rate – 16 cpm
 PEEP – 5
 T n – 360
 FiO2 – 100%
O/E
 Conscious oriented
 Afebrile
 P – 83 bpm
 BP – 120/70 mm of Hg
 CVS - NAD
 SpO2 – 96%
Advice
 Propped up
 Ct all
 Treatment on 05/08/2016 ,
06/08/2016 , 07/08/2016
08/08/2016 , same as on
04/08/2016
 ENT reference was done on
06/08/2016 and
tracheostomy was done on
06/08/2016 under LA and
under all aseptic precautions
 RT feeding was started on
08/08/2016 and diet
reference was done on same
day
S/B Registrar ↓ ICCU / SP ↓ 6 on
09/08/2016 at 8 AM
 GC – moderate
 Afebrile
 P – 95 bpm
 BP – 130/70 mm of Hg
 SpO2 – 100% on Ventilatory
support
 U/O – 1000ml
 RS – clear
 P/A – soft non tender
 CVS – S1S2
 CNS – conscious oriented
Treatment
 D1 Inj piptaz 4.5 gm TDS
 Inj pyridostigmine 80 mg QID
 D10 inj levofloxacin 500mg OD
 Inj neostigmine 0.5mg im SOS
 Inj atropine 0.6cc before inj neostigmine
 D11 inj monocef 1gm BD
 Tab shelcal 1 BD
 Tab azoran 150mg OD
 IVF – 2 point NS
 Inj pantop 40mg OD
 Inj emset 4 mg TDS
09/08/2016 AT 9.30 AM S/B
Senior Physician ↓ 6
 GC – moderate
 Afebrile
 P – 90 bpm
 Chest – clear
 Tracheostomy tube in situ
 P/A – soft
Treatment
 Inj Amino drip OD
 Protein powder
 Inj wymesone (dexamethasone) 4 mg
8hrly
 Tab Azoran 150mg OD
 D2 Inj piptaz 4.5 gm TDS
 D10 Inj levofloxacin 500mg OD
 Tab pyridostigmine 60mg TDS
 Inj emset 4 mg TDS
 10/08/2016 to
18/08/2016
Treatment was same
as on 09/08/2016
 Except
 12/08/2016 tracheal culture & sensitivity
pseudomonas grown
 R – amikacin , aztreonam , cefepime ,
ceftazidime , gentamicin , imipenem ,
piperacillin
 S – polymyxin B
 Day 17 Inj Levofloxacin was stopped on
16/08/2016
 17/08/2016 bladder clamping and bladder
wash started tracheostomy dressing
changed
S/B Neurologist on 18/08/2016
 25yrs /F
 Patient diagnosed case of myasthenia
gravis { AChR antibody - +ve}
 h/o diplopia / weakness of all 4 limbs
since 1year
O/E
 Pt intubated on mechanical ventilation
 SIMV mode with SpO2- 96%
 Occular movements are normal
 Neck weakness - +
 Power – grade 4 in all 4 limbs
 DTR – 2+
 Plantar – b/l ↓
 Pt received plasmapheresis 5
cycles
 Received IVIg 5 days (2gm/kg)
 Patient improving according history
Advice
 Ct tab pyridostigmine 60mg TDS
 Ct tab dexamethasone 4mg TDS
 Ct tab azoran 150mg OD
 Can be considered for repeat IVIg
after 1month if relapse occurs or
difficulty to wean situation
S/B Registrar ↓ ICCU / SP ↓ 6 on
19/08/2016 at 8 AM
 GC – moderate
 Afebrile
 P – 80 bpm
 BP – 130/80 mm of Hg
 SpO2 – 98% on Ventilatory
support
 U/O – 800ml
 RS – clear
 P/A – soft non tender
 CVS – S1S2
 CNS – conscious oriented
Treatment
 Amino drip alternate day
 D11 Inj piptaz 4.5 gm TDS
 Inj wymesone 4mg TDS
 Inj atropine 0.6cc BD
 Tab pyridostigmine 60 mg TDS
 Tab azoran 150mg OD
 Inj pantop 40mg OD
 20/08/2016 same as on
19/08/2016
 21/08/2016 same as on
20/08/2016
 22/08/2016 ,
23/08/2016 same as on
21/08/2016
 Augmentin was started
and piptaz was stopped on
20/08/2016
 Nebulisation with
mucomix was added on
21/08/2016
Patient was discharged on 16/09/2016
on following medicines
 Tab levofloxacin 0.5gm BD
 Tab predmet 16 mg TDS
 Tab distinus (pyridostigmine) 60mg TDS
 Tab azoran 150mg BD
 Protein biscuits
 Follow up after 15 days for work up of thymectomy
Case Discussion
Myasthenia Gravis
 Neuromuscular disorder characterized by weakness and
fatigability of skeletal muscles
 Underlying defect - ↓ in number of available acetylcholine
receptors (AChRs) at neuromuscular junctions due to an
antibody-mediated autoimmune attack
 Myasthenic crisis - Severe weakness of bulbar (innervated
by cranial nerves) and/or Respiratory muscles, enough to
cause inability to maintain adequate ventilation and/or
permeability of upper airways, causing respiratory failure
that requires artificial airway or ventilator support
Standard treatment
Anticholinesterase medication
Thymectomy
Immunosuppression – glucocorticoids or others
Plasmapheresis and IV Immunoglobulins
Rationality
Anticholinesterase medication
 Inj neostigmine 0.5mg BD
 Tab /Inj pyridostigmine 60 mg TDS
 Use – rational
 Improve muscle contraction - Ach released from
prejunctional endings to accumulate and act on
receptors over a larger area, as well as by directly
depolarizing endplate
 Whether combination can be used is not given in any
text book
Rationality
Anticholinergic drugs
 Inj atropine 0.6mg before neostigmine
 Use – rational
 To avoid muscarinic side effects of
anticholinesterase drug
Corticosteroids /
Immunosuppresion
 Inj methyl prednisolone 15- 25mg /day in single dose
to avoid side effects
 Use – Rational
 Inhibit production of Nicotinic Receptor (NR)-
antibodies & may ↑ synthesis of NRs
Dose ↑ stepwise - by 5 mg/d at 2- to 3-day intervals →
marked Clinical improvement or dose of 50 - 60 mg/d
reached
This dose maintained for 1 - 3months
Alternate-day regimen over course of 1 -3 months
Goal is to ↓ dose on off day to zero or to minimal level
Rationality
Tab azathioprine 50mg OD
 Use – Rational
 Previously most commonly used immunosuppressive
agent because of its
 Relative safety
 Long track record
 Therapeutic effect may add to glucocorticoids
effect &/or allow glucocorticoid dose to ↓
Rationality
Plasmapheresis
 Use – rational
 Plasma contains pathogenic antibodies, is
mechanically separated from blood cells
 Course of 5 exchanges (3 - 4 L per exchange) -
Over 10 to 14 day period
 Advantages in Our set up – Comparatively
inexpensive to IVIg but requires expertise
Rationality
Intravenous immunoglobulins
 Use – rational
 Advantage
 Doesn’t require special equipment & Large-bore venous
access
 Ease of administration
 Disadvantage – expensive
 Usual dose is 2 g/kg - over 5 days (400 mg/kg per
day)
Rationality
Antibiotics
 Inj ceftriaxone 1gm iv BD
 Inj levofloxacin 0.5gm iv BD
 Inj piperacillin tazobactum 4.5 gm TDS
 Cap amoxicillin clavulunate 625mg BD
 Use – rational
 Most common cause of crisis is intercurrent
infection
Rationality
Tab thyroxine 100µg OD
 Use – rational
 Hypothyroidism
Inj KCl 10-20meq/hr
 Use – rational
 To treat hypokalemia
Inj calcium gluconate 90mg iv over 10 min post plasma pheresis
 Use – rational
 To treat hypocalcemia associated with plasmapheresis
Not rational
 Prednisolone dose on admission should have been
increased
 Brand names were used
 Respiratory secretions – culture and sensitivity was
done late
 Instead of ceftriaxone they could have used
ceftazidime and cefoperazone
Not rational
 Culture and sensitivity of drugs given was not
done
 Urine & blood culture & sensitivity was not
done
 At some places doses and route of
administration were missing
Next – CCD – DR Swarnank Parmar
Management of myasthenic crisis
 exacerbation of weakness sufficient to endanger life (usually
consists of respiratory failure caused by diaphragmatic &
intercostal muscle weakness)
 Crisis rarely occurs in properly managed patients
 Intensive care units staffed with teams experienced in the
management of MG, respiratory insufficiency, infectious disease,
fluid & electrolyte therapy
 Deterioration could be due to excessive anticholinesterase
medication (cholinergic crisis) - best excluded by temporarily
stopping anticholinesterase drugs
 most common cause of crisis is intercurrent infection
 This should be treated immediately, because the mechanical and
immu-nologic defenses of the patient can be assumed to be
compromised.
 The myasthenic patient with fever & early infection should be
treated like other immunocompromised patients.
 Early & effective antibiotic therapy, respiratory assistance
(preferably noninvasive, using bilevel positive airway pressure), &
pulmonary physiotherapy are essentials of the treatment program
 As discussed above plasmapheresis or IVIg is frequently helpful
in hastening recovery
National Treatment Guidelines
for Antimicrobial Use in Infectious Diseases -
2016

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Clinical case discussion - myasthenia gravis

  • 1. Clinical Case Discussion DR SHINDE VIRAJ ASHOK JUNIOR RESIDENT 3 DEPARTMENT OF PHARMACOLOGY
  • 2. Patient details  Name - Shubhangi Sheshrao Deshbhartar  Age / Sex – 22yrs / F  Reg no – 1226958  Diagnosis – Myasthenia gravis with hypothyroidism
  • 3. S/B AP ↓ ENT 2 on 23/07/2016 Chief complaints  Dysphagia Findings  Oral cavity – WNL  Indirect laryngoscopy - WNL  k/c/o myasthenia gravis on medication since 1 year Advise  Physician opinion
  • 4. S/B Physician ↓ 2 on 23/07/2016 Referred from ENT Chief complaints  k/c/o myasthenia gravis with hypothyroidism since 2 year  Diplopia since 1 ½ year  Slurring speech since 1 ½ month  Weakness of both upper & lower limb since 1 ½ month  Difficulty in deglutition with regurgitation since 8 days O/E  P – 80 bpm  BP – 100/60 mm of Hg S/E  RS & CVS – WNL  CNS – Conscious Oriented  Motor system  Nutrition – average  Power – 5/5  Hand grip – 60%  DTR 2+  Plantar – b/l flexor
  • 5. S/B Physician ↓ 6 on 23/07/2016  No h/o ptosis  Tab thyroxine 100µg  Tab azaron ( azathioprine )50mg OD  Tab omnacortil (Prednisolone)5 mg OD Advice Please admit patient in Female Medicine Ward
  • 6. S/B Lecturer / AP on 23/07/2016 at 2 pm  GC – Moderate  Afebrile  P- 78bpm  RS – Clear Vitals – Stable  Advice Plasmapheresis Nephro call  Same treatment was continued on 24/07/2016 Treatment  1 point DNS  Inj neostigmine 0.5mg BD  Inj atropine 0.6mg before neostigmine  Tab omnacortil (Prednisolone) 5mg OD  Tab thyroxine 100µg OD  ↑↑Tab azaron (azathioprine) 150mg OD
  • 7. S/B Registrar/ SP ↓ med 6 on 25/07/2016  Nasal twang - ++ ,Opthalmoplegia  GC – moderate  Afebrile  P – 90bpm  HS – normal  RS – clear  P/A – soft Investigation  Hb – 7.6gm/dL  TLC – 4000 cells/ mm3  Platelet – 298000/mm3 Treatment  1 point DNS  Inj neostigmine 0.5mg BD  Inj atropine 0.6mg before neostigmine  ↑↑ Tab omnacortil (prednisolone) 40mg OD  Tab thyroxine 100µg OD  Tab azaron (azathioprine)150mg OD  Tab distinon (pyridostigmine) 60mg BD Diagnosis - Myasthenia gravis with exacerbation
  • 8. S/B Lecturer ↓ nephrology on 25/07/2016  Kindly send patient for plasmapheresis  Right sided double lumen HD catheter inserted Advice  HIV - negative  HBsAg – negative  B+ve  Anti HCV  Arrange 5 FFP  Plasma filter  Dialysis tubing
  • 9. S/B Registrar ↓ med 6 on 26/07/2016  GC – moderate  Afebrile  P – 78 bpm  BP – 110/70mm of Hg  RS – NAD  HS – Normal  CNS – NAD Treatment  1 point DNS  Inj neostigmine 0.5mg BD  Inj atropine 0.6mg before neostigmine  Tab omnacortil (prednisolone) 40mg OD  Tab thyroxine 100µg OD  Tab azaron (azathioprine) 150mg OD  Tab distinon (pyridostigmine) 60mg BD
  • 10. 26/07/2016 at 11am – Plasmapheresis - 1  ↓ All Aseptic Precautions double lumen Double Lumen Catheter inserted ↓ local anaesthesia procedure uneventful  Patient had hypotension during Plasmapheresis  1.5 litre plasma removed  5 unit FFP + 1 litre NS infused Advice  Inj monocef (ceftriaxone) 1 gm iv BD  Arrange 5 unit of Fresh Frozen Plasma for next session on 28/07/2016  X ray chest PA view
  • 11. S/B Reg ↓ med 6 / SP ↓ 6 on 27/07/2016  GC – moderate  Afebrile  P – 78 bpm  BP – 110/70mm of Hg  RS – NAD  HS – Normal  CNS – NAD Treatment  1 point DNS  ↑↑ Inj neostigmine 0.5mg TDS  Inj atropine 0.6mg before neostigmine  Tab omnacortil 40mg OD  Tab thyroxine 100µg OD  Tab azaron 150mg OD  Tab shelcal 500mg BD  Tab distinon 60mg BD
  • 12. S/B Lecturer ↓ Nephrology on 28/07/2016 - Plasmapheresis -2 Advice  Plasmapheresis today  2 litre volume exchange  5 point FFP  2 point NS  1 point RL  Inj calcium gluconate …post plasma pheresis  Repeat  CBC  PT/INR  Serum electrolyte
  • 13. S/B Lecturer ↓ Nephrology 28/07/2016 at 3.45 pm  While shifting patient c/o discomfort  P – 150 bpm irregular  SpO2 – 94%  Inj Lasix 20mg iv stat  Inj hydrocortisone 100mg iv stat  Drop in SpO2 to 70%  Hence AMBU bag ventilation done  SpO2 – 98 % achieved  Bipap connected SpO2 – 98% maintained  Semiconscious  ?? Myasthenia Crisis Advice  Shift to Intensive Cardiac Care Uunit & Continue bipap  Urgent Ca++, CBC , PT/INR, ABG
  • 14. Transfer out notes to ICCU on 28/07/ 2016  C/O myasthenia gravis with exacerbation Treatment  1 point DNS  Inj neostigmine 0.5mg TDS  Inj atropine 0.6mg before neostigmine  Tab omnacortil (prednisolone) 40mg OD  Tab thyroxine 100µg OD  Tab azaron (azathioprine) 150mg OD  Inj monocef 1 gm BD  Tab shelcal 500mg OD
  • 15. Receiving notes in ICU 28/07/2016  P – 136bpm  BP – 90/ 60 mm of Hg  SpO2 – 100%  Chest – clear  Dyspnoeic  Sr Ca++ - 10.2  Sr Na+ - 142meq/L  Sr K+ - 2.8 meq/ L  Sr Mg++ - 3.8 meq /L Treatment  2 point NS fast  Inj neostigmine 0.5mg TDS  Inj atropine 0.6mg before neostigmine  Inj monocef 1gm iv BD  Tab omnacortil 40mg OD  Tab thyroxine 100µg OD  Tab azaron 150mg OD  Tab shelcal 500mg BD  Tab distinon 60mg BD  Inj KCl 40meq in 1 point NS over 8hrs
  • 16. S/B Reg ↓ med 6 on 29/07/2016 at 8 AM  GC – moderate  Afebrile  P – 86 bpm  BP – 100/70 mm of Hg  U/O – 800ml  RS – NAD  HS – Normal  CNS – NAD  P /A – soft Treatment  Inj neostigmine 0.5mg TDS  Inj atropine 0.6mg before neostigmine  D5 Tab omnacortil 40mg OD  Tab thyroxine 100µg OD  Tab azaron 150mg OD  Tab shelcal 500mg BD  Tab distinon 60mg BD  D3 Inj monocef 1gm iv BD  pH – 7.4  HCO3- - 22  PCO2 - 25
  • 17. S/B Senior Physician ↓ 6 on 29/07/2016 at 10.40AM  P – 90bpm  Opthalmoplegia ++  No Cyanosis  Chest – Clear  Mild improvement in deglutition Treatment  Inj neostigmine 0.5mg SOS  ↑↑ Tab pyridostigmine 60mg TDS  W/H omnacortil (prednisolone)  D1/3 - Inj methyl prednisolone 500mg OD  Rest ct all
  • 18. Intubation notes 29/07/2016  ↓ All aseptic precautions patient was intubated with ET tube size 7.5 mm AE b/l equal fixed put on ventilatory support SpO2 - 92%  Procedure - uneventful
  • 19. S/B Registrar ↓ ICU 29/07/2016 at 6PM  GC – poor  Unconcious  On ventilatory support  HS – normal  Chest – clear  SpO2 – 92%  Sr T3 – 490 IU  Sr T4 – 18 IU  TSH – 0.01IU Advice  W/H Tab Thyroxine 100µg  Rest ct all
  • 20. S/B MO / Registrar /SP↓ ICCU at 30/07/2016 at 8 AM  GC – moderate  Afebrile  P – 86 bpm  Chest – clear  BP – 110/70mm of Hg  U/O – 650ml  RS – NAD  HS – Normal  CNS – NAD  P/A – soft non tender Treatment  D1 Inj levofloxacin 500mg OD  D4 Inj monocef 1gm iv BD  D2/3 Inj methyl prednisolone 500mg OD  Tab azaron (azathioprine)150mg OD  Inj neostigmine 0.5mg TDS  Inj atropine 0.6mg before neostigmine  Tab pyridostigmine 60mg OD  Tab shelcal 500mg BD
  • 21. S/B Lecturer nephrology at 30/07/2016  c/o myasthenia gravis in myasthenic crisis  Intubated yesterday in view of poor respiratory efforts  P – 80 bpm  BP - 110/70 mm of Hg  B/L AEBE  Concious oriented / moves limb on command Advice  Plasmapheresis today  Arrange 5 @ FFP  Send patient with accompanying resident doctors by12.30PM  High risk plasmapheresis explained to relatives  Sr Ca+ -9.8mg/dL  Sr Mg+ -1.97mg/dL  Sr Na+ -137mEq/L  Sr K+ - 2.6mEq/L
  • 22. S/B SP ↓ 6 at 30/07/2016 at 2 PM  GC- not stable on ventilator  Conscious afebrile  P – 78 bpm  B/L Clear  HS – normal Treatment  ↑↑Tab pyridostigmine 60 mg QID  Rest ct all Investigation  Sr urea -14mg/dL  Sr creat – 0.7mg/dL  T protein – 5.3gm%  T Bilirubin – 0.7mg%  ALP - 44 IU/L  SGOT – 40 IU/L  SGPT – 13 IU/L
  • 23. S/B Lecturer ↓ Nephrology on 30/07/2016 - Plasmapheresis - 3  c/o myasthenia gravis currently in crisis on ventilatory support plasmapheresis 3rd today  No spontaneous breathing on Intermittent Positive Pressure Ventilation Advice  2 litre exchange  5 FFP  2 @ RL  1 @ NS  Inj calcium gluconate 10 cc post plasmapheresis  Post plasmapheresis – there was spontaneous trigerring on ventilator Advice  PT/INR , Sr electrolytes  Tensilon (edrophonium challenge test)  Plan for IVIg  Would offer plasamapheresis on Monday with 5@ FFP & accompanying medicine resident @ 10 AM
  • 24. S/B Registrar ↓ ICCU / SP ↓ 2 on 31/07/2016 at 8 AM  Patient on ventilator  GC – moderate  Afebrile  P – 82 bpm  BP – 120/80 mm of Hg  U/O – 1200ml  RS – clear  P/A – soft non tender  CVS – S1S2  CNS – conscious oriented Treatment  D2 inj levofloxacin 500mg OD  D5 inj monocef 1gm BD  D3/3 inj methylprednisolone 500mg OD  Tab azoran 150mg OD  Inj pyridostigmine 60 mg QID  Inj neostigmine 0.5mg im SOS  Inj atropine 0.6mg SOS before inj neostigmine  Tab shelcal 1 BD  PT/INR - 13/1.11
  • 25. S/B registrar ↓ ICCU / SP ↓ 6 on 1/08/2016 at 8 AM  GC – not stable  Afebrile  P – 80 bpm  BP – 130/90 mm of Hg  U/O – 500ml  RS – clear  P/A – soft non tender  CVS – S1S2  CNS – NAD  SpO2 – 100% Treatment  ↓↓Inj pyridostigmine 60 mg TDS  D3 inj levofloxacin 500mg OD  D6 inj monocef 1gm BD  Inj neostigmine 0.5mg im SOS  Inj atropine 2cc BD  Tab shelcal 1 BD  Tab azoran 150mg OD
  • 26. Transfer notes 11PM at 01/08/2016  Here by transferring out this patient from ward 24 to nephrology for plasmapheresis c/o myasthenia gravis with crisis Treatment  Inj pyridostigmine 60 mg TDS  Inj neostigmine 0.5mg im SOS  Inj atropine 0.6mg SOS before inj neostigmine  D3 inj levofloxacin 500mg OD  D6 inj monocef 1gm BD  Tab azoran 150mg OD
  • 27. S/B Lecturer ↓ Nephrology on 01/08/2016 Plasmapheresis - 4  c/o myasthenia gravis with crisis  BP – 120/ 80 mm of Hg Advice  Plasmapheresis today  2 litre volume exchange  5 point FFP  2 point NS  2 point RL  Inj calcium gluconate …post plasma pheresis
  • 28. S/B Registrar ↓ ICCU / SP ↓ 2 on 02/08/2016 at 8 AM  GC – not stable  Afebrile  P – 86 bpm  BP – 120/80 mm of Hg  U/O – 2000ml  SpO2 – 99%  RS – clear  CVS – S1S2  CNS – conscious oriented  Proximal muscle weakness +  Opthalmoplegia + Treatment  Inj pyridostigmine 60 mg TDS  Inj neostigmine 0.5mg im SOS  Inj atropine 0.6mg SOS before inj neostigmine  Inj rantac 50mg BD  D4 inj levofloxacin 500mg OD  D7 inj monocef 1gm BD  Tab shelcal 1 BD  Tab azoran 150mg OD
  • 29. S/B SP ↓ 6/ SP ICCU at 02/08/2016 at 10 AM  GC – moderate  Conscious  Afebrile  P – 80bpm  Wt – 48 × 2 = 96gm Advice  Plasmapheresis  Free T3 , T4 , TSH  Endocrinologist call  CVTS call Treatment  Ct all  Inj IVIg 20gm/day × 5 days -last day 15gm
  • 30. 03/08/2016 To CVTS surgeon, Sir /madam , kindly evaluate this patient c/o hypothyroidism with myasthenia gravis with thymoma patient is not improving on plasmapheresis . Kindly advice regarding thymectomy Thanking you S/B by Dr Ashish Advice Thymectomy only after GC – improves
  • 31. S/B registrar ↓ ICCU / SP ↓ 6 on 03/08/2016 at 8 AM  Patient on ventilator  GC – not stable  Ventilatory support  Afebrile  P – 90 bpm  BP – 110/70 mm of Hg  RS – clear  P/A – soft non tender  CVS – S1S2  CNS – conscious oriented Treatment  Inj pyridostigmine 60 mg TDS  Inj neostigmine 0.5mg im SOS  Inj atropine 0.6mg SOS before inj neostigmine  D4 inj levofloxacin 500mg OD  D9 inj monocef 1gm BD  Tab shelcal 1 BD  Tab azoran 150mg OD  Plasmapheresis  Nephrologist reference
  • 32. S/B Lecturer ↓ nephrology – 03/08/2016 Plasmapheresis – 5  C/O myasthenia gravis with crisis received 4 plasmapheresis Advice Send patient accompanying medicine resident with 5 @ Fresh Frozen Plasma  Plasma removal – 2 L  Replace with 5 Fresh Frozen Plasma  2 @ RL
  • 33. S/B Registrar ↓ ICCU / SP ↓ 6 on 04/08/2016 at 8 AM  GC – not stable  Afebrile  P – 82 bpm  BP – 120/90 mm of Hg  SpO2 – 100% on Ventilatory support  U/O – 1600ml  RS – clear  P/A – soft non tender  CVS – S1S2  CNS – conscious oriented Treatment  Inj pyridostigmine 60 mg TDS  Inj neostigmine 0.5mg im SOS  Inj atropine 0.6mg SOS before inj neostigmine  D5 inj levofloxacin 500mg OD  D10 inj monocef 1gm BD  Tab shelcal 1 BD  Tab azoran 150mg OD  Plasmapheresis
  • 34. 04/08/2016 Recovery Room To MO/ Registrar Anaesthesia on call kindly call over to evaluate this patient a c/o myasthenia gravis on ventilation not maintaining saturation on A/C mode and opine expertise management Thanking you
  • 35. S/B Anaesthetist on 04/08/2016  C/O myasthenia gravis  Conscious oriented  Afebrile  P – 83 bpm  BP – 120/70 mm of Hg  RS – clear  CVS – S1S2  SpO2- 96% Ventilation  Mode – NC/AC  RR - 16 cycles/min  Positive End Expiratory Pressure - 5  TN – 360  FiO2 -100% Advice  Propped up  Ct ventilatory support  Ct all
  • 36. S/B Registrar ↓ ICCU / SP ↓ 6 on 04/08/2016 at 8 AM  GC – not stable  Afebrile  P – 95 bpm  BP – 130/70 mm of Hg  SpO2 – 100% on Ventilatory support  U/O – 1300ml  RS – clear  P/A – soft non tender  CVS – S1S2  CNS – conscious oriented Treatment  Inj pyridostigmine 60 mg TDS  Inj neostigmine 0.5mg im SOS  Inj atropine 0.5cc before inj neostigmine  D6 inj levofloxacin 500mg OD  D11 inj monocef 1gm BD  Tab shelcal 1 BD  Tab azoran 150mg OD  Tracheostomy tommorrow
  • 37. 04/08/2016 at 1 AM To , Medical officer / registrar Anaesthesia on call, kindly call over to evaluate this patient a c/o myasthenia gravis on ventilation not maintaining saturation on A/C mode and opine expertise management thanks
  • 38. 04/08/2016 at 1.05 AM S/B Anaesthesiologist Registrar A case of myasthenia gravis  Ventilation  Mode VC/AC  Rate – 16 cpm  PEEP – 5  T n – 360  FiO2 – 100% O/E  Conscious oriented  Afebrile  P – 83 bpm  BP – 120/70 mm of Hg  CVS - NAD  SpO2 – 96% Advice  Propped up  Ct all
  • 39.  Treatment on 05/08/2016 , 06/08/2016 , 07/08/2016 08/08/2016 , same as on 04/08/2016  ENT reference was done on 06/08/2016 and tracheostomy was done on 06/08/2016 under LA and under all aseptic precautions  RT feeding was started on 08/08/2016 and diet reference was done on same day
  • 40. S/B Registrar ↓ ICCU / SP ↓ 6 on 09/08/2016 at 8 AM  GC – moderate  Afebrile  P – 95 bpm  BP – 130/70 mm of Hg  SpO2 – 100% on Ventilatory support  U/O – 1000ml  RS – clear  P/A – soft non tender  CVS – S1S2  CNS – conscious oriented Treatment  D1 Inj piptaz 4.5 gm TDS  Inj pyridostigmine 80 mg QID  D10 inj levofloxacin 500mg OD  Inj neostigmine 0.5mg im SOS  Inj atropine 0.6cc before inj neostigmine  D11 inj monocef 1gm BD  Tab shelcal 1 BD  Tab azoran 150mg OD  IVF – 2 point NS  Inj pantop 40mg OD  Inj emset 4 mg TDS
  • 41. 09/08/2016 AT 9.30 AM S/B Senior Physician ↓ 6  GC – moderate  Afebrile  P – 90 bpm  Chest – clear  Tracheostomy tube in situ  P/A – soft Treatment  Inj Amino drip OD  Protein powder  Inj wymesone (dexamethasone) 4 mg 8hrly  Tab Azoran 150mg OD  D2 Inj piptaz 4.5 gm TDS  D10 Inj levofloxacin 500mg OD  Tab pyridostigmine 60mg TDS  Inj emset 4 mg TDS
  • 42.  10/08/2016 to 18/08/2016 Treatment was same as on 09/08/2016  Except  12/08/2016 tracheal culture & sensitivity pseudomonas grown  R – amikacin , aztreonam , cefepime , ceftazidime , gentamicin , imipenem , piperacillin  S – polymyxin B  Day 17 Inj Levofloxacin was stopped on 16/08/2016  17/08/2016 bladder clamping and bladder wash started tracheostomy dressing changed
  • 43. S/B Neurologist on 18/08/2016  25yrs /F  Patient diagnosed case of myasthenia gravis { AChR antibody - +ve}  h/o diplopia / weakness of all 4 limbs since 1year O/E  Pt intubated on mechanical ventilation  SIMV mode with SpO2- 96%  Occular movements are normal  Neck weakness - +  Power – grade 4 in all 4 limbs  DTR – 2+  Plantar – b/l ↓  Pt received plasmapheresis 5 cycles  Received IVIg 5 days (2gm/kg)  Patient improving according history Advice  Ct tab pyridostigmine 60mg TDS  Ct tab dexamethasone 4mg TDS  Ct tab azoran 150mg OD  Can be considered for repeat IVIg after 1month if relapse occurs or difficulty to wean situation
  • 44. S/B Registrar ↓ ICCU / SP ↓ 6 on 19/08/2016 at 8 AM  GC – moderate  Afebrile  P – 80 bpm  BP – 130/80 mm of Hg  SpO2 – 98% on Ventilatory support  U/O – 800ml  RS – clear  P/A – soft non tender  CVS – S1S2  CNS – conscious oriented Treatment  Amino drip alternate day  D11 Inj piptaz 4.5 gm TDS  Inj wymesone 4mg TDS  Inj atropine 0.6cc BD  Tab pyridostigmine 60 mg TDS  Tab azoran 150mg OD  Inj pantop 40mg OD
  • 45.  20/08/2016 same as on 19/08/2016  21/08/2016 same as on 20/08/2016  22/08/2016 , 23/08/2016 same as on 21/08/2016  Augmentin was started and piptaz was stopped on 20/08/2016  Nebulisation with mucomix was added on 21/08/2016
  • 46. Patient was discharged on 16/09/2016 on following medicines  Tab levofloxacin 0.5gm BD  Tab predmet 16 mg TDS  Tab distinus (pyridostigmine) 60mg TDS  Tab azoran 150mg BD  Protein biscuits  Follow up after 15 days for work up of thymectomy
  • 48.
  • 49. Myasthenia Gravis  Neuromuscular disorder characterized by weakness and fatigability of skeletal muscles  Underlying defect - ↓ in number of available acetylcholine receptors (AChRs) at neuromuscular junctions due to an antibody-mediated autoimmune attack  Myasthenic crisis - Severe weakness of bulbar (innervated by cranial nerves) and/or Respiratory muscles, enough to cause inability to maintain adequate ventilation and/or permeability of upper airways, causing respiratory failure that requires artificial airway or ventilator support
  • 50. Standard treatment Anticholinesterase medication Thymectomy Immunosuppression – glucocorticoids or others Plasmapheresis and IV Immunoglobulins
  • 51.
  • 52. Rationality Anticholinesterase medication  Inj neostigmine 0.5mg BD  Tab /Inj pyridostigmine 60 mg TDS  Use – rational  Improve muscle contraction - Ach released from prejunctional endings to accumulate and act on receptors over a larger area, as well as by directly depolarizing endplate  Whether combination can be used is not given in any text book
  • 53. Rationality Anticholinergic drugs  Inj atropine 0.6mg before neostigmine  Use – rational  To avoid muscarinic side effects of anticholinesterase drug
  • 54. Corticosteroids / Immunosuppresion  Inj methyl prednisolone 15- 25mg /day in single dose to avoid side effects  Use – Rational  Inhibit production of Nicotinic Receptor (NR)- antibodies & may ↑ synthesis of NRs Dose ↑ stepwise - by 5 mg/d at 2- to 3-day intervals → marked Clinical improvement or dose of 50 - 60 mg/d reached This dose maintained for 1 - 3months Alternate-day regimen over course of 1 -3 months Goal is to ↓ dose on off day to zero or to minimal level
  • 55. Rationality Tab azathioprine 50mg OD  Use – Rational  Previously most commonly used immunosuppressive agent because of its  Relative safety  Long track record  Therapeutic effect may add to glucocorticoids effect &/or allow glucocorticoid dose to ↓
  • 56. Rationality Plasmapheresis  Use – rational  Plasma contains pathogenic antibodies, is mechanically separated from blood cells  Course of 5 exchanges (3 - 4 L per exchange) - Over 10 to 14 day period  Advantages in Our set up – Comparatively inexpensive to IVIg but requires expertise
  • 57. Rationality Intravenous immunoglobulins  Use – rational  Advantage  Doesn’t require special equipment & Large-bore venous access  Ease of administration  Disadvantage – expensive  Usual dose is 2 g/kg - over 5 days (400 mg/kg per day)
  • 58. Rationality Antibiotics  Inj ceftriaxone 1gm iv BD  Inj levofloxacin 0.5gm iv BD  Inj piperacillin tazobactum 4.5 gm TDS  Cap amoxicillin clavulunate 625mg BD  Use – rational  Most common cause of crisis is intercurrent infection
  • 59. Rationality Tab thyroxine 100µg OD  Use – rational  Hypothyroidism Inj KCl 10-20meq/hr  Use – rational  To treat hypokalemia Inj calcium gluconate 90mg iv over 10 min post plasma pheresis  Use – rational  To treat hypocalcemia associated with plasmapheresis
  • 60. Not rational  Prednisolone dose on admission should have been increased  Brand names were used  Respiratory secretions – culture and sensitivity was done late  Instead of ceftriaxone they could have used ceftazidime and cefoperazone
  • 61. Not rational  Culture and sensitivity of drugs given was not done  Urine & blood culture & sensitivity was not done  At some places doses and route of administration were missing
  • 62. Next – CCD – DR Swarnank Parmar
  • 63. Management of myasthenic crisis  exacerbation of weakness sufficient to endanger life (usually consists of respiratory failure caused by diaphragmatic & intercostal muscle weakness)  Crisis rarely occurs in properly managed patients  Intensive care units staffed with teams experienced in the management of MG, respiratory insufficiency, infectious disease, fluid & electrolyte therapy  Deterioration could be due to excessive anticholinesterase medication (cholinergic crisis) - best excluded by temporarily stopping anticholinesterase drugs
  • 64.  most common cause of crisis is intercurrent infection  This should be treated immediately, because the mechanical and immu-nologic defenses of the patient can be assumed to be compromised.  The myasthenic patient with fever & early infection should be treated like other immunocompromised patients.  Early & effective antibiotic therapy, respiratory assistance (preferably noninvasive, using bilevel positive airway pressure), & pulmonary physiotherapy are essentials of the treatment program  As discussed above plasmapheresis or IVIg is frequently helpful in hastening recovery
  • 65.
  • 66.
  • 67.
  • 68. National Treatment Guidelines for Antimicrobial Use in Infectious Diseases - 2016