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Abstract Details

Evaluation of Measures for Ocular Manifestations in Myasthenia Gravis
Neuromuscular and Clinical Neurophysiology (EMG)
P1 - Poster Session 1 (11:45 AM-12:45 PM)
11-002
To compare different assessments for ocular myasthenia gravis (MG) symptoms.

MG commonly presents with diplopia and ptosis. There are limited studies assessing the validity and performance of multimodal ocular MG assessments. 

 

We enrolled patients with confirmed MG in the US and Canada. Patients completed questionnaires including demographics, the ocular component of the Myasthenia Gravis Impairment Index (OMGII), the diplopia questionnaire, and symptom satisfaction. Raters assessed extra-ocular movements (EOM), ptosis, and diplopia in different positions; centers with neuro-ophthalmologists quantified ocular alignment using the prism cover test. Patients with a second visit were reassessed with all measures and patient impression of change.  We assessed correlations between the different assessments and compared mean scores between patients satisfied vs. dissatisfied with current symptoms.

We enrolled 52 subjects (37% female; mean age 67± 13 years); mean disease duration 8 years± 9.6, 15 (29%) had generalized disease.  Thirty-two subjects had 2 visits, for a total of 84 assessments. In 56 (71.8%) assessments, diplopia was reported, and in 41 (52.6%) ptosis.  On examination, 32.1% had abnormal EOM, 55.1% diplopia and 70.5% ptosis.  The OMGII total score correlated moderately (r 0.51-0.69, p < 0.05) with most assessments. The OMGII ptosis and diplopia questions correlated highly with ptosis (r 0.73-0.83, p < 0.05), diplopia on exam (0.75-0.86, p < 0.05) and prism assessment (r:0.64-0.71, p<0.05). EOM exam had low correlation (0.36, p <0.05) with OMGII and diplopia questionnaires (r 0.29, p < 0.05). In 41 (49%) of assessments, subjects reported satisfaction with symptoms, and this was associated with OMGII, diplopia questionnaire, diplopia and prism examination scores, but not to ptosis or EOM examination.

We found varying strengths in relationship between patient-reported outcomes and examination. Patient-satisfaction was associated with patient-reported measures and diplopia/prism scores during examination, but not with ptosis or EOM examination.

 

Authors/Disclosures
Carolina Barnett Tapia, MD
PRESENTER
Dr. Barnett Tapia has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Argenx. Dr. Barnett Tapia has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen. Dr. Barnett Tapia has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for UCB. Dr. Barnett Tapia has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Barnett Tapia has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NMD pharma. Dr. Barnett Tapia has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Barnett Tapia has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for argenx. Dr. Barnett Tapia has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Janssen. Dr. Barnett Tapia has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. Dr. Barnett Tapia has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for argenx. The institution of Dr. Barnett Tapia has received research support from Department of defense. The institution of Dr. Barnett Tapia has received research support from Muscular dystrophy canada. The institution of Dr. Barnett Tapia has received research support from MgNet. The institution of Dr. Barnett Tapia has received research support from NIH. The institution of Dr. Barnett Tapia has received research support from Cartesian. The institution of Dr. Barnett Tapia has received research support from Alexion. Dr. Barnett Tapia has received intellectual property interests from a discovery or technology relating to health care.
Catherine Lawton, MD Ms. Lawton has nothing to disclose.
Meg Mendoza, PhD Dr. Mendoza has nothing to disclose.
Ali G. Hamedani, MD, MHS (Hospital of the University of Pennsylvania) Dr. Hamedani has received personal compensation in the range of $500-$4,999 for serving as a Consultant for ArgenX. Dr. Hamedani has received personal compensation in the range of $0-$499 for serving as a Consultant for LoQus23. The institution of Dr. Hamedani has received research support from NIH. The institution of Dr. Hamedani has received research support from Biogen. The institution of Dr. Hamedani has received research support from Biohaven.
Gil I. Wolfe, MD, FAAN (Univ. At Buffalo, SUNY) Dr. Wolfe has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Grifols. Dr. Wolfe has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Alexion. Dr. Wolfe has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for ArgenX. Dr. Wolfe has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for UCB/Ra. Dr. Wolfe has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen. Dr. Wolfe has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Cartesian. Dr. Wolfe has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Grifols. The institution of Dr. Wolfe has received research support from UCB/Ra. The institution of Dr. Wolfe has received research support from Immunovant. The institution of Dr. Wolfe has received research support from Roche. Dr. Wolfe has received personal compensation in the range of $0-$499 for serving as a Advisor with FDA Advisory Panel for Cellular, Tissue and Genetic Therapies.
Joome Suh, MD (Brigham and Women's Hospital) Dr. Suh has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. Dr. Suh has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for argenx.
Mark J. Kupersmith, MD, FAAN (Icahn School of Medicine At Mount Sinai) Dr. Kupersmith has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Legal firms. Dr. Kupersmith has stock in SafeTherapeutics m,LLC. The institution of Dr. Kupersmith has received research support from NEI, NYEEI FDN, Shulman Family NAION fund. Dr. Kupersmith has received intellectual property interests from a discovery or technology relating to health care.