This study compared the clinical manifestations of 71 patients with ocular myasthenia gravis (MG) to those with generalized MG. Patients with generalized MG had a higher rate of other autoimmune diseases and required long-term steroid treatment more often than those with ocular MG alone. Both groups experienced similar ophthalmic symptoms. The study recommends regular eye exams for all MG patients due to risks from autoimmune diseases and long-term steroid use.

1. Comparison of Clinical Manifestations
between Patients with
Ocular Myasthenia Gravis and
Generalized Myasthenia Gravis
Roh HS, Lee SY, Yoon JS
Korean J Ophthalmol 2011;25(1):1-7
Journal Reading
dr. Ersifa Fatimah
dr. M. Saiful Islam, SpS(K)

2. Myasthenia Gravis
Autoimmune disease characterized by
muscular fatigue due to defective
neuromuscular transmission
with the levator palpebralis and
extraocular muscles preferentially
affected
2

3. MG patients MG patients
> 75% MG px
present with
90% will
visual
generalize
complaints
within 3 years
50% of px with
ocular
manifestation
develop 80% will
generalized generalize
weakness within within 2 years
6 months
3

4. Immune-related disorder (DM
1, SLE, Sjogren, Graves, Hashimoto, RA, MS)

Blurry
vision, tearing, pain, photophobia, reduced
visual acuity, decreased tear
film, inflammatory soft
tissue, exophthalmos, etc
Long-term steroid therapy
MG:
Diplopia / ocular motility
defects, ptosis
Ocular
manifestation
4

5. Previous studies of MG have not focused on
ophthalmic manifestations other than
ptosis, diplopia, and those caused by the long-
term of MG
No known studies comparing the ophthalmic
features & complications between patients with
ocular MG those of generalized MG
5

6. Materials & Methods
Px diagnosed with MG, Jan 1995 - Dec 2007, minimum 1 year follow-up period
Database of Dept Neurology & Ophthalmology Yonsei University College of Medicine
Data, Diagnosis, & Classification
Clinical evaluation, confirmatory
Demographic, ophthalmic Osserman score
diagnostic test (>/1 from Anti-
symptom & sign, associated Gr. I = Ocular gr.
Ach Rec Ab, RNST, Neostigmine
autoimmune dis., results of
test, Pyridostigmine), CT thorax Gr. II-IV = Generalized gr.
diagnostic test
+ contrast
Analysis: SPSS ver 12.0; Pearson chi-square, Fisher’s exact test; Wilcoxon rank sum
test; significant if p-value < 0.05
6

7. Results & Discussion
• A rare disease, it is often difficult to analyze a large patient population
• Median follow up period: 39 months (12-105 months)
• Range age at disease onset: 3-71
• 65% px develop initial symptoms at age <50 ys.
• Mean time interval from the onset of ocular MG to generalization: 3.5 ys
7

8. Bimodal pattern of age of onset in both genders : early-onset & late-onset
Incidence rates peaking 60 - 80 ys, male predominance in the older age group
(Allen et al, 2010)
Mean age of disease
onset 40.1 ys (male) &
42.8 ys (female) 8

9. • A significant number of patients reported various ophthalmic symptoms;
all of which may be related to the muscular weakness caused by MG.
• Symptoms were observed equally in the ocular & generalized MG groups.
• 7% of generalize MG group had no ptosis / diplopia
9

10. Diagnostic Tests
The basic diagnostic methods for MG had lower sensitivities in the
ocular MG group compared to those in the generalized MG group.
10

11. The incidence of total autoimmune disease was higher in patients in the
generalized MG group (p=0.721)
Consistent with previous study by Christensen et al, 1995
11

12. • Anticholinesterase drugs improve symptoms of MG in nearly all ocular and generalized
MG patients, but the therapeutic effects are limited. Thus, most patients require
additional immunosuppressive treatment such as corticosteroid treatment
• Although still controversial, there are several studies which have insisted that systemic
steroid treatment in ocular MG patients can prevent disease progression to generalized
MG.
12

13. Long-term corticosteroid effect
An important method for preventing complications in patients receiving
corticosteroids is to limit the total steroid dose.
13

14. One way to reduce the steroid dose is
by performing early thymectomy during the course of treatment.
Thymectomy is accepted as an effective treatment for MG, for which it
is considered a first line immune treatment.
Thymectomy was performed in 65% of generalized MG patients, and
the majority was able to reduce steroid dosage while continuing
symptom improvement.
14

15. Advice for Neurologist
Neurologists may not detect ocular symptoms and signs other than ptosis
and diplopia or may not be aware of the ophthalmic complications that
can result from combined autoimmune diseases and steroid treatment 
MG patients with ophthalmic signs and symptoms may not receive
immediate proper ophthalmic management.
It is important that patients with ocular MG or generalized MG with
ophthalmic symptoms should undergo regular eye examinations.
Careful attention must be given to generalized MG patients because they
have a higher risk of ophthalmic and other problems associated with
systemic autoimmune disease and long-term treatment of MG than do
ocular MG patients
15

16. Study Limitations
• Small sample
• Selection bias
• Cannot quantitatively compare the efficacies
of the treatment to severity of the symptoms
due to fluctuations in disease status &
treatment response
16

17. Conclusion
• MG patients may experience less common eye
problems  can impair quality of life and should not
be neglected.
• Common autoimmune diseases further supporting
ophthalmic complications. In generalized MG, the
incidence of autoimmune disease is higher and more
variable than that in ocular MG patients;
therefore, generalized MG patients should be screened
for ophthalmic complications.
• Long term steroid treatment in MG patients may also
cause complications must be taken into careful
consideration
17

18. Critical Appraisal
• Study design: Retrospective
• P = Patients with MG
• I / C = Ocular MG & Generalized MG
• O = difference in clinical manifestation
(dx, tx, symptom & sign, related disease)

20. Best
“study designs”
for ...
20

21. Validity
• Representative sample
Sample size 71, inclusion/exclusion (?)
• Allocation
Random (-) Concealed (-)
Comparable (+)
• Maintenance
Adequate follow up period (+)
Equal management (-)
Drop out (?) Change group (?)
• Measurement
Blind (?) Objective +/-

22. Importance
• Statistical significance  available p-value
• Dx: Sensitivity/Specificity (+)
• Tx: ?
• Related disease, complication: ?
• The research was not intended to determine
which one is better
• Open data  viewer can analyze

23. Applicability
• Patient/ case (+), facility (+)

24. 24

25. Thank You
25