Treatment Refractory Ocular Symptoms in Myasthenia Gravis: Clinical and Therapeutic Profile (P6.445)

Objective: To review clinical characteristics of patients with treatment resistant ocular symptoms in myasthenia gravis (MG). Background: Most MG patients (85%) develop ocular symptoms over the course of the disease. Despite many therapeutic options available for management of MG, some patients remain resistant to standard therapies. Design/Methods: We performed a retrospective chart review of MG patients with treatment resistant ocular symptoms. Treatment resistance was defined as persistent diplopia or ptosis despite prednisone and at least one additional immunosuppressive agent. After IRB approval, patient’s charts were reviewed to abstract demographic, clinical, diagnostic and therapeutic information. Results: We identified 4 patients with acetylcholine receptor antibody positive MG with treatment resistant ocular symptoms attending our clinics. The median age of MG onset was 26 years (range of 23 to 61). Three patients had ocular symptoms at onset and developed generalized MG at some time along the course of their illness. The fourth case continued to have ocular MG. High dose steroid was started immediately after the symptom onset in 2/4 patients, 3 months after onset in one patient and 8 years after onset in one patient. The median time interval between the onset of MG to the refractory ocular symptoms was 13 years (range of 3 to 25). Initial partial response was reported with pyridostigmine in 3/4 patients, high dose steroids in 2/4 patients, other immunosuppressive agents in 1/3 patients, IVIG in 2/3 patients. None of the 3 patients who had plasmapheresis (PLEX) showed any significant improvement. Ocular symptoms persisted in all 4 patients despite all therapies. Conclusions: MG patients with treatment refractory ocular symptoms are mostly younger at disease onset and had earlier ocular symptoms. Ocular symptoms were resistant to PLEX and IVIG. Disclosure: Dr. Sharma has nothing to disclose. Dr Pasnoor has nothing to disclose. Dr. Dimachkie has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Dr. Dimachkie is on the speaker’s bureau or is a consultant for Baxalta, Catalyst, CSL Behring, Mallinckrodt, Novartis and NuFactor. He has received grants from Alexion, Biomarin, Catalyst, CSL-Behring, FDA/OPD, GSK, MDA, NIH, Novartis, Orphazyme and TM. Dr. Barohn has nothing to disclose. Dr. Jawdat has nothing to disclose. Dr. Glenn has nothing to disclose. Dr. Farmakidis has nothing to disclose. Dr. Jabari has nothing to disclose.