Laporan Kasus: Myasthenia Gravis

Laporan kasus
MYASTHENIA GRAVIS
MOCH HASYIM
Epidemiology
 Frequency
 Annual incidence in US- 2/1,000,000 (E)
 Worldwide prevalence 1/10,000 (D)
 Mortality/morbidity
 Recent decrease in mortality rate due to advances in treatment
 3-4% (as high as 30-40%)
 Risk factors
 Age > 40
 Short history of disease
 Thymoma
 Sex
 F-M (6:4)
 Mean age of onset (M-42, F-28)
 Incidence peaks- M- 6-7th decade F- 3rd decade
Pathophysiology
 In MG, antibodies are directed toward the
acetylcholine receptor at the neuromuscular junction
of skeletal muscles
 Results in:
 Decreased number of nicotinic acetylcholine
receptors at the motor end-plate
 Reduced postsynaptic membrane folds
 Widened synaptic cleft
Pathophysiology
 Anti-AChR antibody is found in 80-90% of patients
with MG
 Proven with passive transfer experiments
 MG may be considered a B cell-mediated disease

 Antibodies
 T-cell mediated immunity has some influence

 Thymic hyperplasia and thymomas are
recognized in myasthenic patients
THE NEUROMUSCULAR JUNCTION
Etiology
• The Unknown cause.
• Defect in the transmission of nerve
impulses to muscles.
• Antibodies against acetyl choline receptors
at the NMJ.
• Thymic abnormalities.
Factors that can worsen myasthenia
gravis
• Fatigue
• Illness
• Stress
• Extreme heat
• Some medications — such as beta
blockers, calcium channel blockers,
quinine and some antibiotics
OSSERMAN Classification
1. Class I Any ocular muscle weakness
2. Class II Mild weakness other than ocular

IIa Predominantly limb,axial, or both
IIb Predominantly orpharyngeal/respiratory
3. Class III Moderate weakness other than ocular
IIIa Predominantly limb,axial, or both
IIIb Predominantly orpharyngeal/respiratory
4. Class IV Severe weakness other than ocular
IVa Predominantly limb,axial, or both
IVb Predominantly orpharyngeal/respiratory
5. Class V Intubation with/without ventilation

NEUROLOGIC CONDITIONS MIMICKING MYASTHENIA GRAVIS
CONDITION SIGNS AND SYMPTOMS
ALS Asymmetric muscle weakness and atrophy

Botulism Generalized limb weakness
Guillain-Barré syndrome Ascending limb weakness
Inflamm. muscle disorders Proximal symmetric limb weakness
Lambert-Eaton syndrome Proximal symmetric limb weakness
Multiple sclerosis Bilateral internuclear ophthalmoplegia
Periodic paralysis Intermittent generalized muscle weakness
Thyroid disease
Congenital myasthenic syndromes
Brainstem syndromes/encephalitis
DRUGS PRECIPITATING MYASTHENIA
Anti-infective Agents Cardiovascular Agents Other Agents
Aminoglycosides Propranolol Chloroquine

Ampicillin Verapamil Corticosteroids
Ciprofloxacin Quinidine “d-penicillamine”
Erythromycin Procainamide Phenytoin
Imipenem Propafenone Mydriatics
Kanamycin Acebutolol Trihexyphenidyl
Pyrantel Practolol Interferon
Timolol Trimethadione
Oxyprenolol
flowchart for the management of myasthenia gravis
Figure 2. A treatment flowchart for the management of myasthenia gravis. IVIG, intravenous
immunoglobulin; PE, plasma exchange Dr Sathasivam is a Consultant Neurologist at The Walton
Centre NHS Foundation Trust, Liverpool
Medical management
• Anticholinesterase drugs. (pyridostigmine
bromide)
• Immunosuppressive therapy.
(prednisolone)
• Plasmapharesis.
• Intravenous immunoglobulin (IVIG)
therapy.
• Thymectomy.
AChE inhibitor
 Inhibit the enzymatic elimination of acetylcholine, increasing its
concentration at the post synaptic membrane
 Gives partial improvement in most myasthenic pts although
complete improvement in very few pts
◦ Pyridostigmine (mystinon) most widely used
 Adult dose: - starts with 60mg 4 times daily, increase up to 120
mg 4 times daily
Long acting drug can be used at bed time
Starts working in 15-30 minutes and lasts 3-6 hours but response
varies with individual
 Caution
Check for cholinergic crisis
 Others: Neostigmine Bromide
dose 7.5-30 mg average of 15 mg 6th hrly
1.5mg im for 2hrly and 0.5mg iv
Immunomodulating therapies
Glucocorticoid therapy
◦ Prednisone is the most commonly used corticosteroid
 Should be given in a single dose to minimize the side
effects
 Initial dose is 15-25 mg/d, increase by 5mg at 2-3days
interval until marked clinical improvement achieved or
50-60mg/day is reached
 Maintain the same effective dose for 1-3 months, then
modify to alternate day regimen over the additional 1-3
months
 Taper the dose and asses the effective minimum dose
 Close monitoring is necessary
Intravenous Immunoglobulin Therapy
 The administration of intravenous

immunoglobulin (IVIG) serves as an alternate
mode of therapy to plasmapheresis.
 This procedure is especially helpful when
vascular access is a problem.
 Intravenous immunoglobulin is given as a dose
of 2 g/kg in divided doses over 2 to 5 days.
 Intravenous immunoglobulin therapy is a
relatively safe treatment method and has few
adverse effects, though headache, chills, and
fever have been reported in some patients.
 Other rare adverse events include aseptic
meningitis and renal failure.
Plasmapheresis
 Plasma exchange, or plasmapheresis, is an effective

means of therapy but is transient in its response (2-8
wks)
 Useful when treating patients in myasthenic crises or
those in preparation for surgery and at the start of
immunosuppressive therapy.
 The goal of this therapeutic intervention is to remove
the circulating immune complexes and AchR-Ab.
 Patients usually undergo a 2-week course of 5 to 6
exchanges (2-3.5L each).
 Removed plasma is replaced with albumin and saline
 Risks involved in this treatment include infection, DVT,
fluid imbalance and hypercoagulation.
thymectomy
 Surgical Intervention-introduced by blalock
 Surgical removal of thymoma- If a patient has a thymoma, it
should clearly be removed
 Thymectomy as a treatment for MG
 85% of pts experiences improvement after thymectomy,
of these 35% achieves drug free remission
 Clinical improvement is typically delayed by 6 months to
1 year after surgery, but maximum effect occurs after 3
years and offers the long term benefit.
 Should be carried out in all pts with generalized MG who
are between puberty and 55 years of age.
 Pts with anti MuSK anti body may not respond
 Preferred electively and not during acute crisis
 Transsternal thoracotomy is preferred and allows for
maximal exposure to ensure that all thymic tissue is
removed at the time of surgery.
Complications of MG
 Respiratory failure
 Dysphagia
 Complications secondary to drug
treatment
 Long term steroid use
 Osteoporosis, cataracts, hyperglycemia,
HTN
 Gastritis, peptic ulcer disease
 Pneumocystis carinii
LAPORAN KASUS
Tn. A ( 17 tahun )
MRS di RSHS tgl 27-7-2015
KX : pendertia datang ke poli saraf dengan keluhan tidak bisa
membuka mata
AX : penderita mengeluh tidak bisa membuka mata sejak 1 th
yl , keluhan bertambah berat sejak 1 mgg sebelum kepoli
saraf, mata kabur (-), penglihatan ganda (-), pandangan gelap (-),
RPD : (-)
RPK : (-)
Alergi (-)
PD : T 120/80, N , 80x/mnt, RR 20x/mnt, T0 36,7 0C
CT Thymus : pembesaran kelenjar thymus
DX : myasthenia gravis dengan pembesran kelenjar Thymoma
DD: thymic limphoid hyperplasia
TX : - IVFD RL 1000 cc / 24 j
- diet TKTP
- lab : Hb,Hct, Leuko, Trombo, , Na, K, Cl, Ur/Cr, SGOT/SGPT,
Alb, AGD
- Rencana Thymectomy
- persiapan darah PRC 4 kolf
Lab : Hb 15,4; Hct 45; Leuco 9000; Trombo 304.000; Ureum 22;
Creatinin 0,6; SGOT 23; SGPT 27;
RO Thorax : Tidak tampak metastase intra pulmunal,

cardiomegali (-)
CT Thymus : pembesaran kelenjar thymus
Post Operasi : tgl 13-8-2015
• DPO : propofol 50mg/jam
• Kesadaran : E4M6V ET
• T 140/70; N 105x/mnt; RR 20x/mnt; SaO2 100%
• Ventilator : CPAP PS 5; peep 5 ; FiO2 50%
• Lab : Hb 15,4; Hct 45; Leuco 9000; Trombo 304.000; Ureum 22;
Creatinin 0,6; SGOT 23; SGPT 27;
• AGD : pH 7,35/pCo2 33/pO2 83/HCO3- 18/BE -7/SaO2 97%
• Terapi :
• IVFD 1200/24 jam
• Pasang NGT
• Nutrisi Parenteral
• Ranitidine 1 amp/12 jam
• Tramadol 2 amp in 500cc RL/8 jam
• Ceftriazone 2 gr/24 jam
Riwayat Perjalanan Penyakit
POLI SARAF BANGSAL SARAF ICU
27/8/2015 DX: Tumor mediastinum Post operasi
1 thn sebelum MRS mengeluh ec Thymoma thymectomy
kelopak mata jatuh, DD: Thymic lymphoid
Di poli saraf tidak bisa membuka
hyperplasia Puasa
mata, kabur(-), lihat dobel (-),
IVFD : RL 1500 cc/24j
pandangan gelap (-)
RPD (-)
TX: IVFD RL1000/24 j Pasang NGT
RPK (-) Diet TKTP Diet sonde
Alergi(-) Persiapan operasi Ceftriaxone 1gr/12 j
T 120/80, N80x/mnt, RR20x/mnt, Ranitidine 1 amp / 12 j
T 36,7 C 13/8 / 2015 Tramadol 2 Amp drip
Anemis -/-, ict-/- Operasi thymectomy in 500 cc / 24 jam
C/P dbn MO 30 ug/KgBB/24 j
Abd dbn Post operasi rawat ICU Prostigmin 10 Amp +
CT thymus : pembesaran kel SA 5 Amp / 24 jam
thymus, Observasi tanda vital
DX: Tumor mediastinum ec
Thymoma
DD: Thymic lymphoid hyperplasia
ICU Hari–1
BP HR RR
M
200 200 A
S
U
K
160 160 I
50
C
U
120 120 40
80 80 30
40 40 20
20 20 10
7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 1 2 3 4 5 6 7
S : (-) A P: F : entramix 1800 kal

O: CNS : DPO Miathenia Gravis A: MO 20ug/kgbb/jam
CVS : TD 142/76 mmHg, HR 120x/mnt, RR 28 x/mnt,
Post op Tymectomy S : propofol 50 mg/jam
T0 36,70C
Thoraks : luka operasi dada anterior T:-
ventilator : CPAP ; Peep 5 ; FiO2 50% H: headup 450
PS 8 ; SaO2 100%
C / P dbn U: ranitidin 1amp/12 jam
Abd : GIT : distensi (-) G: -
GUT : dbn TX : ceftriazone 1g / 12Jam
DR : hb 10,2, Leuko 10000, SOFA SCORE : 2
Agd : 7,35/33/83/18/-7/97% POD : 2
ranitidine / 12 jam
GDS 110-130 Ur 104 Cr 1.1 CPIS : - mestinon 60 mg/8jam
Na/K/Cl 135/5.6/83 ,
UO 4 cc/kg/j,, BK -1000 cc
ICU Hari–1
BP HR RR
M
200 200 A
S
U
K
160 160 I
50
C
U
120 120 40
80 80 30
40 40 20
20 20 10
7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 1 2 3 4 5 6 7

T0 36,70C
PS 8 ; SaO2 100%
GUT : dbn TX : ceftriazone 1g / 12Jam
DR : hb 10,2, Leuko 10000, SOFA SCORE : 2
Agd : 7,35/33/83/18/-7/97% POD : 2 ranitidine / 12 jam
GDS 110-130 Ur 104 Cr 1.1 CPIS : - mestinon 60 mg/8jam
Na/K/Cl 135/5.6/83 ,
UO 4 cc/kg/j,, BK -1000 cc
ICU Hari–3
BP HR RR
P
A
S
200 200 A
N
G
C
V
160 160 C
50
120 120 40
80 80 30
40 40 20
20 20 10
7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 1 2 3 4 5 6 7

T 36,70C
0

PS 8 ; SaO2 100%
GUT : dbn TX : ceftriazone 2 g / 8 jam
DR : hb 9,4, Leuko 10000,
Agd : 7,44/34/108/23/0/98 ranitidine / 12 jam
Prostigmin : SA = 10:5 / 24 j
UO = 40cc/j BK = 1300CC flumucyl 200mg/8j
metylprednisolon 125mg/12j
ICU Hari–5
P
L
BP HR RR A
S
M
A
200 200 P
H
E
R
E
160 160 S
50 I
S
I
120 120 40
80 80 30
40 40 20
20 20 10
7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 1 2 3 4 5 6 7

O: CNS : DPO Miathenia Gravis A: MO 10mg/kgbb/jam
CVS : TD 123/76 mmHg, HR 120x/mnt, RR 28 x/mnt, T0
36,70C
PS 8 ; SaO2 100%
DR : hb 10,2, Leuko 10000,
Agd : 7,35/33/83/18/-7/97% ranitidine / 12 jam
HB 6,4/HT20/L8000/TR234000 flumucyl 200mg/8j po
Na132/k5,6/cl105/ca4,73/mg1,9 pros :sa = 10: 5 amp/24 j
UO 2 cc/kg/j,, BK 1800 cc
transfusi PRC 2 kolf
ICU Hari–7
P
L
BP HR RR A
S
M
A
200 200 P
H
E
R
E
160 160 S
50 I
S
2
120 120 40
80 80 30
40 40 20
20 20 10
7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 1 2 3 4 5 6 7

36,70C
PS 6 ; SaO2 100%
DR : hb 9,8/HT29/L9700/TR289000
Agd : 7,41/28/128/17/-6/99 ranitidine / 12 jam
pros :sa = 10: 5 amp/24
UO 4 cc/kg/j,, BK 2000 cc Flumucyl 200mg/8j
methylprednisolon 125mg/12j
ICU Hari–9
P
L
BP HR RR A
S
M
A
200 200 P
H
E
R
E
160 160 S
50 I
S
3
120 120 40
80 80 30
40 40 20
20 20 10
7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 1 2 3 4 5 6 7

36,70C
PS 6 ; SaO2 100%
DR : hb 10,7, Leuko 18000,ht31,tr333.000
GDS 110-130 Ur 104 Cr 1.1 pros :sa = 10: 5 amp/24
Na/K/Cl 135/5.6/83 , flumucyl 200mg/8j
UO 3 cc/kg/j,, BK 986 cc
ICU Hari–11
P
L
BP HR RR A
S
M
A
200 200 P
H
E
R
E
160 160 S
50 I
S
4
120 120 40
80 80 30
40 40 20
20 20 10
7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 1 2 3 4 5 6 7

36,10C
SaO2 100%
DR : hb 10,2, Leuko 10000,tr324000
GDS 130 Ur 104 Cr 1.1 sa : pros = 5: 10/24jam
Na/K/Cl 135/5.6/83 flumucyl 200mg/8j
UO 4 cc/kg/j, BK 1200 cc
ICU Hari–12
P
L
BP HR RR A
S
M
A
200 200 P
H
E
R
E
160 160 S
50 I
S
5
120 120 40
80 80 30
40 40 20
20 20 10
7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 1 2 3 4 5 6 7

36,70C
: t-piece 10 lt H: headup 450
C / P dbn
Abd : GIT : distensi (-) U: ranitidin 1amp/12 jam
GUT : dbn G: -
DR : hb 10,2, Leuko 10000, TX : ceftriazone 2 g / 8 jam
Agd : 7,35/33/83/18/-7/97%
GDS 110 Ur 104 Cr 1.1 ranitidine / 12 jam
Na/K/Cl 135/5.6/83 , pros :sa = 10: 5 amp/24
UO 4 cc/kg/j, BK 980 cc flumucyl 200mg/8j
ICU Hari–16
BP HR RR
E
X
200 200 T
U
B
A
S
160 160 I
50
120 120 40
80 80 30
40 40 20
20 20 10
7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 1 2 3 4 5 6 7

36,70C
t-piece 8 lt H: headup 450
SaO2 100%
DR : hb 10,2, Leuko 10000,
GDS 110-130 Ur 104 Cr 1.1 pros :sa = 10: 5 amp/24
Na/K/Cl 135/5.6/83 , flumucyl 200/8j
UO 4 cc/kg/j, BK 300 cc
ICU Hari–20
BP HR RR
200 200
PASIEN PINDAH
160 160
50
RUAANGAN
120 120 40
80 80 30
40 40 20
20 20 10
7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 1 2 3 4 5 6 7

36,70C
nasal canul 3lpm sao2 98% H: headup 450
C / P dbn
Abd : GIT : distensi (-) U: ranitidin 1amp/12 jam
GUT : dbn G: -
DR : hb 10,2, Leuko 10000, TX : ceftriazone 2 g / 8 jam
Agd : 7,35/33/83/18/-7/97%
GDS 110 Ur 104 Cr 1.1 ranitidine / 12 jam
Na/K/Cl 135/5.6/83 , Sa : prost = 5: 10 /24j
UO 4 cc/kg/j,BK 400 cc Flumucyl 200 mg/8j
DISKUSI
Pada kasus ini, terapi utama Myasthenia Gravis sudah
sesuai dengan short-term immunotherapy yaitu
plasmapheresis
Pada pasien ini dilakukan plasmapheresis :
volume processed = 5898 ml
As volume used = 556 ml
Volume subtituted = 2097 ml
Volume Extracted = 2097
Subtituted PCT = 100%
DISKUSI
Pada kasus ini, terapi utama Myasthenia Gravis sudah
sesuai dengan short-term immunotherapy yaitu
plasmapheresis
Pada pasien ini dilakukan plasmapheresis :
volume processed = 5898 ml
As volume used = 556 ml
Volume subtituted = 2097 ml
Volume Extracted = 2097
Subtituted PCT = 100%
Plasmapheresis juga berguna untuk memperbaiki

DISKUSI
Saat ini terapi yang dikenal untuk pasien myasthenia
gravis adalah: 1.Antikolinesterase, 2.Imunosupresan
dan kortikosteroid, 3.Plasmapharesis (TPE),
4.Intravena imunoglobulin (IVIG), dan 5.Thymectomy.
Plasmapharesis (TPE) pada myasthenia gravis adalah
prosedur memisahkan sel darah merah dari plasma
yang mengandung antibodi yang merusak asetilkolin
dan diganti dengan cairan pengganti seperti FFP,
albumin, koloid atau kristaloid.
DISKUSI
 Plasmapharesis sangat bermanfaat untuk
mengatasi keadaan krisis myasthenia, sebagai
persiapan sebelum thymectomy dan juga
mempercepat pemulihan pasien dari kondisi
paralisis dan gangguan gagal nafas akibat krisis
myasthenia. Dibandingkan IVIG, TPE terbukti lebih
ekonomis dengan hasil yang memuaskan bila
dilakukan secara dini.
 Saran : pada kasus ini sebaiknya dilakukan
plasmapharesis sebelum dilakukan tymectomy
TERIMA KASIH

Laporan Kasus: Myasthenia Gravis

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 MG may be considered a B cell-mediated disease

 T-cell mediated immunity has some influence

2. Class II Mild weakness other than ocular

5. Class V Intubation with/without ventilation

CONDITION SIGNS AND SYMPTOMS

ALS Asymmetric muscle weakness and atrophy

Anti-infective Agents Cardiovascular Agents Other Agents

Aminoglycosides Propranolol Chloroquine

 The administration of intravenous

 Plasma exchange, or plasmapheresis, is an effective

RO Thorax : Tidak tampak metastase intra pulmunal,

S : (-) A P: F : entramix 1800 kal

S : (-) A P: F : entramix 1800 kal

S : (-) A P: F : entramix 1800 kal

Thoraks : luka operasi dada anterior T:-

S : (-) A P: F : entramix 1800 kal

S : (-) A P: F : entramix 1800 kal

S : (-) A P: F : entramix 1800 kal

S : (-) A P: F : entramix 1800 kal

S : (-) A P: F : entramix 1800 kal

S : (-) A P: F : entramix 1800 kal

S : (-) A P: F : entramix 1800 kal

Plasmapheresis juga berguna untuk memperbaiki

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