Abstract Details

Title Clinical Features and Treatment Responses of Ocular Myasthenia Gravis in Japan

Topic Muscle Disease/Neuromuscular Junction

Presentation(s) P01 - (-)

Poster/Presentation
Number 115

Objective

Background BACKGROUND: Although immunotherapy for oMG is still controversial, various treatments can improve the prognosis of oMG patients.

Design/Methods DESIGN/METHODS: Data was obtained from the Japan MG registry established in 2012. Clinical features were assessed as defined by the MG Foundation of America. Quantitative MG scores were divided into ocular and generalized components (oQMGs and gQMGs).

Results RESULTS: Among 676 registered cases, 607 MG patients who were followed for at least 2 years were entered into the present study. We studied the 123 patients (20%) who had oMG (age 60±16 years, M:F = 54:69). During the treatment course 81 patients developed both ptosis and diplopia, 36 had ptosis alone, and 6 had diplopia alone. We classified oMG patients into 2 groups: those having ptosis alone (29%; group P) and those having diplopia with or without ptosis (71%; group D). The disease severity scores were 2.6±0.7 and 4.6±1.7 in oQMGs (groups P and D, p<0.001) and 2.8±2.1 and 2.5±1.5 in gQMGs (groups P and D, P=0.4). In groups P and D, 56% and 83% of patients were positive for anti-AChR antibodies (P=0.002), respectively. Treatment included prednisolone (42%), high-dose intravenous methylprednisolone (mPSL; 19%), calcineurin inhibitors (23%), thymectomy (25%), anticholinesterase inhibitors (80%), and topical naphazoline (24%). Responses rates were 53% (low-dose prednisolone), 91% (mPSL), 75% (calcineurin inhibitors), 66% (anticholinesterase inhibitors), and 63% (topical naphazoline). There were no differences in treatment regimens or responses between the two groups. Postinterventional status showed remission or minimal manifestation in 75% and 68% of group P and D patients (P=0.4).

Conclusions CONCLUSIONS: Further treatment strategy will be necessary to improve the prognosis of oMG patients.

Authors/Disclosures
PRESENTER	No disclosure on file
Hiroyuki Murai, MD (International University of Health and Welfare)	Dr. Murai has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Murai has received personal compensation in the range of $500-$4,999 for serving as a Consultant for argenx. Dr. Murai has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Murai has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Chugai Pharmaceutical. Dr. Murai has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis. The institution of Dr. Murai has received research support from Japan Blood Products Organization.
Ludwig Kappos, MD, FAAN (RC2NB, University Hospital Basel)	Dr. Kappos has nothing to disclose.
	No disclosure on file
Yuriko Nagane, MD, PhD (Hanamaki General Hospital, Neurology Dept)	No disclosure on file
Masayuki Masuda	Masayuki Masuda has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Alexion pharma, Argenix pharma, Aasahi kasei pharma, UCB, Takeda pharma.
	No disclosure on file
Shingo Konno, MD	Dr. Konno has nothing to disclose.
	No disclosure on file
Shunya Nakane, MD	No disclosure on file
Norihiro Suzuki	No disclosure on file
Kimiaki Utsugisawa, MD, PhD (Hanamaki General Hospital)	Dr. Utsugisawa 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. Utsugisawa has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB pharma. Dr. Utsugisawa has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Janssen pharma. Dr. Utsugisawa has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Viela Bio. Dr. Utsugisawa has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Chugai. Dr. Utsugisawa has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Mitsubisi Tanabe pharma. Dr. Utsugisawa has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Dr. Utsugisawa has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for argenx. Dr. Utsugisawa has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion phama. Dr. Utsugisawa has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Japan Blood Products Organization. Dr. Utsugisawa has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB.

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