1. Eye Findings in Myasthenia Gravis: Diagnosis, Evaluation, and Treatment
Sophia M. Chung, M.D.
Depts of Ophthalmology &
Neurology and Psychiatry
Saint Louis University School of Medicine

2. Ocular Myasthenia Gravis
>90% patients w/MG have eye finding
Ptosis (droopy lid)
Diplopia (double vision)
50% patients’ initial presentation of MG
Most convert to generalized MG
Within 2 years

3. Ocular Myasthenia Gravis
Pure ocular disease occurs in 20% MG
Later age
Men > women

4. Generalized Myasthenia (Grob et al. ‘81)

5. Ocular Myasthenia (Grob et al. ‘81)

6. Ocular Myasthenia Gravis
24 yo man complains of droopiness of left upper lid-variable throughout the day

7. Ocular Myasthenia Gravis
Ptosis
Isolation
Company of double vision
Variable
Often asymmetric
Fatigable
Worsens later in the day

8. Ocular Myasthenia Gravis

9. Ocular Myasthenia Gravis
Patient photos

10. Ocular Myasthenia Gravis
Ptosis (droopy eyelid)
Cogan’s lid twitch
Patient asked to look up after looking down
Eyelid briefly over-elevated and appears to be waving

11. Ocular Myasthenia Gravis
Video ptosis from MG

12. Myasthenia Gravis Double vision Variable May involve: One eye muscle
Multiple eye muscles
All eye muscles

13. Ocular Myasthenia Gravis
Patient photos

14. Ocular Myasthenia Gravis
Diagnosis
Sleep test
Ice test

15. Ocular Myasthenia Gravis
Before ice test
After ice test

16. Ocular Myasthenia Gravis
Weakness of eyelid muscles
Nearly always present

17. Ocular Myasthenia Gravis
Diagnosis: anti-acetylcholine receptor Ab
Present in 80-90% generalized MG
Present in 50% ocular MG
Level has no correlation with disease activity

18. Ocular Myasthenia Gravis
Thyroid function tests
20% MG patients has concurrent thyroid disease
Anti-nuclear antibody
Erythrocyte sedimentation rate

19. Ocular Myasthenia Gravis
Tensilon test
Test dose
Bolus
Response within 30 sec
Duration 2-5 minutes
Look for change

20. Ocular Myasthenia Gravis
Patient photos

21. Ocular Myasthenia Gravis
Repetitive stimulation test
Positive 20-30% ocular MG
Single fiber EMG
Eyelid muscles or forehead
85-100% sensitivity

22. Ocular Myasthenia Gravis
Chest CT
Thymomas present in 15% all MG patients
Considered rare-uncommon in ocular MG
Presence warrants surgery

23. Myasthenia Gravis CT chest Thymoma 10-15% of MG
(of thymomas, 50% have MG)
>age 30, males
Thymus hyperplasia 60-80%
younger age, females
Anti-Ach Rec Ab % of the time w/thymoma

24. Ocular Myasthenia Gravis
No randomized clinical trials for pure ocular MG
Treatment based on clinical experience, retrospective trials

25. Ocular Myasthenia Gravis
Treatment
Withdraw medications that might exacerbate MG
Long list: cardiac drugs, antibiotics, anti-epileptics, psychiatric drugs
Most common: aminoglycosides and beta-blockers

26. Ocular Myasthenia Gravis
Pyridostigmine (Mestinon)
Less effective in pure ocular MG
50% patients respond
Ptosis responds better than diplopia
Side effects: gastrointestinal
Contraindications: bradycardia, reactive airway disease, BPH

27. Ocular Myasthenia Gravis
Corticosteroids
Start low (5-10mg) and increase slowly until symptoms improve
Maintain resolution
Slow taper
Recurrence common despite slow taper
Improvement of ptosis & diplopia 72-96% patients

28. Ocular Myasthenia Gravis
Corticosteroids
Side effects
Mood swings -Cataract
Sleep disorders -GERD
Hyperglycemia -Osteoporosis
Weight gain -Hypertension

29. Ocular Myasthenia Gravis
Azathioprine
Mycophenolate mofetil
Cyclosporine A
Tacrolimus
Cyclophosphamide
Rituximab
Etanercept

30. Myasthenia Gravis Plasmapharesis (respiratory failure)
Human Immunoglobulin
Thymectomy
Referral to Neurologist

31. Ocular Myasthenia Gravis
Patient photos

32. Myasthenia Gravis: Thymectomy
Data for generalized MG helpful early in disease
Promotes long term clinical improvement under the age of 50
Unclear role with pure ocular MG
Always indicated with the presence of thymoma
50% chance of malignancy

33. Patient photos

34. Ocular Myasthenia Gravis
Conclusions
>90% patients w/MG have eye finding
Ptosis and diplopia
50% patients’ initial presentation of MG
Most convert to generalized MG

35. Ocular Myasthenia Gravis
Conclusions
20% MG is purely ocular
Diagnosis often more challenging
Treatment usually requires 2 or more drugs