Myasthenia gravis

Clinical features :
Fluctuating ptosis is the
characteristic presentation
Autoimmune disorder
Antibodies to acetylcholine
receptors
Easy fatiguability
Life threatening symptoms
dysphagia, dyspnoea

Ptosis and Diplopia

Ptosis worsen in
Prolonged
upgaze

Pupil always normal

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Kupersmith MJ, Ying G.

Ocular motor dysfunction
and ptosis in ocular
myasthenia gravis: effects
of treatment. Br J
Ophthalmol 2005;89:1330
4.

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Ptosis
myogenic

Diagnosis confirm :
edrophonium chloride
testing
acetylcholine
receptor antibody
assay
EMG
ice-pack test

Physical examination:
Margin-reflex distance
Vertical palpebral
fissure height
Upper eyelid crease
position
Levator function
bells phenomenone

Ocular MG / systemic
MG (+)

Pyridostigmin
Prednison
Azatioprine

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Taken From : Manual

systematic eyelid
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surgery

Sensitivity and specificity of

testing for ocular myastenia
gravis

Taken from :
Your Smith
date here
and nesi

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AChE inhibitor
Inhibit the enzymatic elimination of acetylcholine, increasing its
concentration at the post synoptic membrane
Gives partial improvement in most myasthenic pts although complete
improvement in very few pts
Pyridostigmine 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 5060mg/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

 Patients may have transient worsening of

MG symptoms during the first 2 to 3 weeks
of prednisone therapy.
Patients should be warned of these
potential adverse effects at the initial
stages of therapy and reassured them.
Significant improvement is often seen
after a decreased antibody titer which is
usually 1-4 months

Azathioprine
The most commonly used drug to treat patients with
MG until now .
It allows tapering of steroid dosage and reduces
some of the adverse effects of steroid therapy.
starting dose of azathioprine is 50 mg daily for the
first week (test dose), and then the dose is titrated
up to a maximum of 2- 3 mg/kg body weight daily in
two or three divided doses.
The most common adverse effects are neutropenia
and liver function abnormalities.

Cyclosporine and tacrolimus -Calcineurin inhibitors

These agents are usually prescribed for patients who
have failed to respond to combination therapy with
prednisone and azathioprine and those who cannot
tolerate azathioprine.
Beneficial effects are more rapid than
azathioprine
dose cyclosporine 3-6mg/d and tacrolimus 0.1mg/kg
in two divided doses
The most important adverse effects are nephrotoxicity
and hypertension.

CyclophosphamideIn general,
cyclophosphamide is used only in
refractory cases
Cyclophosphamide therapy may be started
at 25 mg daily and gradually increased up to
a maximum of approximately 2- 5
mg/kg/day .
An increased incidence of hemorrhagic
cystitis accompanies the use of this
medication in some patients

Mycophenolate Mofetil
Inhibits the purine synthesis by the de novo
pathway and so inhibits the proliferation of
lymphocytes ,not other cells
Currently is being used as an adjunct to
corticosteroids due to relative less side effecs
Recent trials in patients with MG have shown this
medication to provide significant benefit.
The standard daily dosage is 1 g to 2 g in two
divided doses.
Side effects-occasional diarrhea rarely leucopenia
very useful in long-term treatment
But high cost.

Prophylaxis of the complications of

immunosuppression

Osteoporosis prevention -Measure bone density before

treatment and yearly while on treatment. Start calcium and
vitamin D supplements. Bisphosphonates may reduce bone loss
associated with the chronic use of glucocorticoids.
Cardiovascular risk - Risk factor modification , advice to stop
smoking, start an exercise program and manage hypertension.
Peptic ulcer prevention -Helicobacter screening and
prophylactic treatment with proton pump inhibitors or H2
antagonists .
Infection prophylaxis - Use of inactivated vaccines such as
influenza is recommended. Live vaccines are contraindicated. A
chest X-ray should be performed prior to treatment. More
specific testing for tuberculosis may be indicated depending on
history and chest X-ray results.
Malignancy prevention -Skin cancer rates are increased in
patients using azathioprine. A full yearly dermatological survey
is recommended. Regular cervical smears are recommended.
Eye protection may also limit cataract development .

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.

BlepharoPtosis

Defenisi
Turunnya kelopak
mata atas dibawah
kedudukan yang
normal dan dapat
menutupi aksis
visual atau tidak
dan terjadinya
dapat unilateral
atau bilateral

NORMAL Posisi
KELOPAK MATA
ATAS
2 mm dibawah
limbus atau
antara limbus
atas dan pusat
pupil

 Bukan merupakan suatu diagnosis, tapi

merupakan suatu tanda
Dihubungkan dengan adanya kelainan
pada muskulus levator palpebra/muskulus
muller yang berfungsi untuk mengangkat
kelopak mata
Dapat mengenai semua umur
Bisa kongenital atau didapat
Penyebabnya banyak

Anatomy

Klasifikasi

AAO section 7 page 201

Klasifikasi

Neurogenik

Myogenik

Pseudoptosis

Post enucleation socket syndrom

Dermatochalasis
Hemifacial spasm
Lid retraction mata kontra lateral
Essential blefrospasm

Ptosis kongenital

Perkembangan otot levator palpebra yang

jelek (myogenik)

Dengan fungsi rektus superior normal

Dengan fungsi rektus superior buruk
Merupakan suatu syndrom (blefarofimosis)
Ptosis sinkinetik

 Ptosis aponeurotik : akibat stretching,

dehisced (pecahnya) atau disinsersi
aponeurosis levator

Pemeriksaan Fisik

Anamnesa
Mulai terjadinya ptosis
Perubahan-perubahan yang terjadi -> apakah
stabil atau semakin memberat
Riwayat keluarga
Riwayat operasi & trauma
Riwayat penyakit kelopak yang pernah diderita
Riwayat penyakit sistemik yang berhubungan
dengan ptosis
Mempelajari foto-foto terdahulu untuk
konfirmasi lama dan berat ptosis

Pemeriksaan Oftalmologi

BELLS PHENOMENON
Penderita diminta menutup matanya dengan kuat,
lalu pemeriksa membuka kelopak mata atas untuk
melihat apakah bola matanya bergulir keatas
(+) bila bola mata bergulir ke atas

(-) bila bola mata tidak bergulir keatas

 Penderita melihat pada posisi primer,

lakukan hisberg test. Kemudian diukur jarak
antara margo palpebra superior dan reflek
cahaya di pupil
Normal : 2-4 mm
Untuk menentukan derajat ptosis
Ringan MRD 1,5 2mm
Sedang MRD 3 mm
Berat MRD 4mm

Margin Reflek Distance

(MRD)1

Margin Reflek Distance

(MRD)2
Penderita melihat pada posisi primer,
lakukan hisberg test. Kemudian diukur
jarak antara margo palpebra inferior dan
reflek cahaya di pupil

Fissura Palpebra
Jarak antara margo palpebra superior dan
inferior pada posisi primer
Normal : 9-10 mm

Levator Action
Penderita diminta melihat kebawah maksimal
Tangan kanan pemeriksa memegang
penggaris dan meletakkan titik nol pada
margo palpebra superior. Tangan kiri
pemeriksa menekan otot frontal agar tidak
mengangkat kelopak. Lalu penderita diminta
melihat keatas maksimal dan dilihat margo
palpebra superior.
Normal 14-16 mm

Margin Limbal Distance

(MLD)
Penderita melihat dalam posisi primer,
kemudian diminta melirik keatas maksimal
kemudian diukur jarak antara margo
palpebra superior dan limbus inferior
Normal : 9-10 mm

Lid Lag

Penderita disuruh melihat keatas

kemudian melihat kebawah. Dilihat
apakah ada lag palpebramenunjukkan
ketidak mampuan m. Levator palpebra
untuk melakukan relaksasi, karena adanya
fibrosis pada m. Levator palpebra

Lid Crease
Lipatan kulit pada kelopak mata atas
Normalnya 7mm (8-10 mm)
5mm (10-12 mm)

Tensilon Test
Bila curiga Miasthenia Gravis
10 mg tensilon disuntikkan iv, mula-mula
2 mg disuntikkan dalam 15-30 detik
Kemudian bila dalam 1 menit tidak ada
reaksi, disuntikkan lagi 8 mg secara
perlahan-lahan. Bila ptosis menghilang
myastenia gravis

Ice Pack-Test
Sekantong es batu diletakkan pada
kelopak mata penderita selama 2 menit.
Jika membaik Myastenia gravis

PHENILEPHRINE TEST

Teteskan phenylephrin 10 % pada mata

ptosis bila ptosis menghilang, ptosis
disebabkan karena muskulus muller

Penatalaksanaan

Pada penatalaksanaan ptosis

ada 3 hal yang perlu
ditentukan :

Klasifikasi & Evaluasi Klinis

Tehnik Operasi

Fasanella servat
Advancement aponeurosis levator
Levator reseksi
Fascia lata suspension

Fungsi Levator

Buruk : Fascialata Suspension

Sedang
Reseksi
Baik
Levator
Istimewa : Fasanella servat (bila penyebab
muskulus muller); pengencangan
aponeurosis

Reseksi Levator

Bagaimana mengukur
besarnya reseksi levator ?
{MLD normal MLD ptosis} x 3
Bila LA < 8 mm; hasil ditambahkan 1-2
mm
Bila LA > 10 mm; hasil dikurangkan 1-2
mm

Folow up

Penilaian pasca operasi

MRD
Komplikasi:
Keratitis exposure
Lid lag
Lagoftalmus
Over/under koreksi
Entro/ectropion
Hilang bulu mata
Symblefaron
Infeksi
Hemorrhage

Keberhasilan Operasi Ptosis

Berhasil Bila :
Untuk ptosis unilateral : tinggi kelopak
mata atas pasca operasi harus sama atau
berbeda 1 mm dengan tinggi kelopak
mata atas yang normal
Untuk ptosis bilateral : MRD 2-4 mm dan
perbedaan MRD kanan dan kiri kurang dari
sama dengan 1 mm

Aponeurotic
Myogenic
Acquired Ptosis

Neurogenic
Mechanical

Traumatic
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70

Edrophonium cloride
testing.
a small test dose of 2 mg (0.2 mL)
edrophonium is injected intravenously, and
the patient is observed for 60 seconds.
If the symptoms disappears or decrease (for
example, the eyelid elevates or motility
improves),
the test result is considered positive and can
be discontinued. If no response is elicited,
additional doses of 4 mg, up to a total of 10
mg, are given.
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