S32 Copyright 2019 The Korean Society of Neuro-Ophthalmology http://neuro-ophthalmology.co.kr
Ocular Myasthenia Gravis: Strategies for the Personalized Therapy
최재환
양산부산대학교병원 신경과
ISSN: 2234-0971
대한안신경의학회지: 제9권 Supplement 1
Clin Neuroophthalmol 9(Suppl 1):S32-35, April 2019
Ocular myasthenia gravis:
Strategies for the personalized therapy
양산부산대학교병원 신경과 최 재 환
Contents
Case review
Non-medical therapy
Medical therapy
Surgical therapy
Current treatment options of MG
1) No treatment
2) Mechanical treatments: lid crutch, patching 3) Anticholinesterase therapy
4) Oral immunosuppressive therapy 5) Intravenous immunoglobulin 6) Plasmapheresis
7) Thymectomy
8) Monoclonal antibody therapy: rituximab 9) Ptosis/eye muscle surgery
Non-medical therapy (1)
Ocular Myasthenia Gravis: Strategies for the Personalized Therapy •
최재환Clin Neuroophthalmol 9(Suppl 1):S32-35, April 2019 http://neuro-ophthalmology.co.kr S33
Non-medical therapy (1) Non-medical therapy (2)
Medical therapy
Topical naphazoline
Pyridostigmine
Prednisolone
Mycophenolate mofetil
Azafrin
Others: cyclosporine, tacrolimus, rituximab, IVIG
Topical naphazoline
Sympathomimetic drug with α2 activity
Selectively increase the tone of Müller muscle
Widen the palpebral fissure
Cosmetic and functional effects for mild to moderate myopathic ptosis or partial Horner syndrome
Overall effects of naphazoline
Muscle Nerve 2011
최재환
• Ocular Myasthenia Gravis: Strategies for the Personalized Therapy
Clin Neuroophthalmol 9(Suppl 1):S32-35, April 2019 S34 http://neuro-ophthalmology.co.kr
Pyridostigmine
A mainstay of therapy of ocular MG
Advantage: safety, variable dosing, relative quickness in determining efficacy
Typically more effective for ptosis than diplopia
60-120mg q4-q8 hours
SR form 180-360mg 1-2x/day
Caution: abdominal cramping, diarrhea, bradycardia
Pyridostigmine alone failed to cause remission of the disease
Oral immunosuppressive therapy
There are no RCTs providing the efficacy of any of the common immuno- suppressive therapies in ocular MG.
Multiple retrospective series, clinical experience, and experience with
generalized MG all lead to a reasonable conclusion that immunosuppressive therapy is effective in ocular MG.
Considerable factors: age, medical comorbidities, side effect, cost….
Prednisolone
A commonly used first choice of oral immunosuppressive drug
Advantage: wide availability, low cost, well-known side effect, reasonable efficacy, reduce the frequency of deterioration to generalized MG
Disadvantage: worsening of disease in patients with preexisting diabetes mellitus, hypertension, and osteoporosis
Start High Dose?
N = 55
Resolution in primary gaze diplopia in 74% at 1 month (Only 7% in pyridostigmine group)
Br J Ophthalmol 2005
Start Low Dose? - EPITOME
Muscle Nerve 2016
Start Low Dose? - EPITOME
Muscle Nerve 2016
N = 11 (9 completed 16-week of double-blind therapy)
Treatment failure incidence was 100% in the placebo vs. 17% in the
prednisolone group.
Ocular Myasthenia Gravis: Strategies for the Personalized Therapy •
최재환Clin Neuroophthalmol 9(Suppl 1):S32-35, April 2019 http://neuro-ophthalmology.co.kr S35
Mycophenolate mofetil Mycophenolate mofetil
Dose: 1000mg once or twice daily
Advantage: ease of use, minimal adverse effects, a study of its use in ocular MG
Disadvantage: monthly monitoring of CBC, greater cost than prednisolone, longer time needed for improvement than prednisolone.
Azathioprine
Advantage: cost, long-term experience with excellent knowledge of adverse effects, a study of its use in ocular MG
Disadvantage: long time to onset
Other treatments
Cyclosporine A, Tacrolimus, Methotrexate, Rituximab, Cyclophosphamide: no head-to-head trials of these drugs
IVIG, Plasmapheresis: usually are not required for ocular MG
Surgical therapy: Thymectomy
A recent meta-analysis of thymectomy in ocular MG notes a remission rate of 50% and recommends thymectomy be considered in patients with ocular MG
Thymectomy in patients without thymoma
• Effective in generalized MG
• Generally not be recommended in ocular MG
