Abstract Details

Title Case Series: Adding Intravenous Immunoglobulin Therapy in Steroid Refractory Ocular Myasthenia Gravis.

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P3 - Poster Session 3 (12:00 PM-1:00 PM)

Poster/Presentation
Number 1-011

Objective Case series adding Intravenous immunoglobulin (IVIG) therapy in steroid refractory ocular myasthenia gravis

Background

IVIG is used in acute myasthenia gravis flares but insufficient data exists for ocular myasthenia gravis (OMG)^1,2. Randomized control trials comparing IVIG vs. placebo have not shown statistically significant improvement in Quantitative Myasthenia Gravis (QMG) Scores^3,4. The EPITOME⁵ trial compared chronic prednisone versus placebo in OMG and demonstrated efficacy by QMG, NEI Visual Function Questionnaire-25 (NEI-VFQ-25)⁶ and Myasthenia Gravis Quality of Life 15 Scale (MG-QoL-15). Immunosuppressive therapies are limited by side effects and delayed onset of action in patients with OMG. We hypothesized that adding IVIG to patients not responsive to prednisone therapy would improve myasthenic symptoms.

Design/Methods We report a single-institution case series of patients with OMG treated with IVIG. Eight patients, ages 51-78 years were included. NEI-VFQ-25 and MG-QoL-15 were administered retrospectively for pre- and post-treatment conditions after 6 months of IVIG. Prednisone was weaned according to symptoms. IVIG was dosed 1g/kg monthly after loading dose of 2g/kg for all patients.

Results After IVIG, reported quality of life improved significantly in all patients. In MG-QOL-15, scores improved by mean 22 points (p = .000; 95% CI 13.6 – 30.4). NEI-VFQ-25 scores improved by mean 42.4 points (p = .002; 95% CI 26.44-58.36). Prednisone dosing decreased by mean 7.38 milligrams (p = 0.068; 95% CI -.72 – 15.47) in the 6 months following IVIG initiation. NEI VFQ 25 scores improved by mean 42.4 points (p = .002, 95% CI 26.44-58.36). In all cases, IVIG was well-tolerated without serious adverse events.

Conclusions

Patients in this series demonstrated durable improvement in quality of life with IVIG at 6 months. IVIG was well-tolerated and allowed weaning of prednisone therapy. These results preliminary support IVIG use for ocular symptoms in myasthenia gravis resistant to moderate dose prednisone, however prospective trials are needed.

Authors/Disclosures
Hannah C. Machemehl, MD (Austin Neuromuscular Center) PRESENTER	No disclosure on file
Jamie Morrell	No disclosure on file
Sami L. Khella, MD, FAAN (Presbyterian Med Ctr/Dept of Neuro)	Dr. Khella has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Ionis. Dr. Khella has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Ionis. Dr. Khella has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Pfizer. Dr. Khella has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alnylam. Dr. Khella has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Eidos.

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