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

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

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

Generalized Myasthenia (Grob et al. ‘81)

Ocular Myasthenia (Grob et al. ‘81)

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

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

Ocular Myasthenia Gravis

Ocular Myasthenia Gravis Patient photos

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

Ocular Myasthenia Gravis Video ptosis from MG

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

Ocular Myasthenia Gravis Patient photos

Ocular Myasthenia Gravis Diagnosis Sleep test Ice test www.globalspine.net/best_position_sleeping.html www.smartertravel.com/travel-advice

Ocular Myasthenia Gravis Before ice test After ice test

Ocular Myasthenia Gravis Weakness of eyelid muscles Nearly always present www.yorku.ca/earmstro/journey/images/facial.gif

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

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

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

Ocular Myasthenia Gravis Patient photos

Ocular Myasthenia Gravis Repetitive stimulation test Positive 20-30% ocular MG Single fiber EMG Eyelid muscles or forehead 85-100% sensitivity www.eorthopod.com/images/ContentImages/spine

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

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 +95-100% of the time w/thymoma

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

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

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

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

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

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

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

Ocular Myasthenia Gravis Patient photos

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

Patient photos

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

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