Abstract Details

Title Therapeutic Dilemma in Generalized Myasthenia Gravis and Good's Syndrome Overlap

Topic Muscle Disease/Neuromuscular Junction

Presentation(s) P02 - (-)

Poster/Presentation
Number 195

Objective

Background BACKGROUND: Good's syndrome (thymoma with immunodeficiency) is a rare cause of combined B and T cell immunodeficiency in adults.Good's syndrome can be uncommonly associated with myasthenia gravis (MG).Immunosuppressive therapy for MG in patients with Good's syndrome is complicated by development of recurrent opportunistic infections.

Design/Methods DESIGN/METHODS: Case Report.

Results RESULTS: Case: 65 year old male with a history of hypertension presented with episodes of double vision, fatigue,dysphagia and generalized weakness.Anti acetylcholine antibodies were positive. Repetitive nerve stimulation showed decremental response.CT chest showed thymoma for which he underwent resection and was placed on high dose oral steroids,mycophenolate and mestinon. 4 months after starting treatment he presented with shortness of breath,chest X-ray showed a reticulonodular pattern and further work revealed histoplasmosis.Mycophenolate was stopped but he was on a tapering course of steroids when he developed diarrhea.Further work-up revealed CMV colitis.At this time he was completely weaned off steroids but he continued to have recurrent pneumococcal infections.8 months later he developed disseminated candidal infection.Immunological studies showed hypogammaglobulenemia affecting all antibody types. There was cutaneous anergy to intra dermal antigen challenge.Flow cytometry revealed reduced mature circulating B cells,reduced CD4 count and reversal of CD4:CD8 ratio.Bone marrow biopsy showed reduced pre-B cell lineage.Patient has remained stable on monthly IVIG which is used to treat both MG and Good's syndrome.

Conclusions CONCLUSIONS: Recurrent opportunistic infections in the setting myasthenia (with or without immunosuppressive therapy) and thymoma should raise the suspicion of Good's syndrome. Periodic long term intravenous immunoglobulin can be a treatment option for both MG and Good's syndrome.

Authors/Disclosures
Virgilio D. Salanga, MD, FAAN (Cleveland Clinic - Florida) PRESENTER	No disclosure on file
	No disclosure on file
Raghav Govindarajan, MD, FAAN (HSHS St. Elizabeth Medical Group)	Dr. Govindarajan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for MT pharma. Dr. Govindarajan 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. Govindarajan 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. Govindarajan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Catalyst. Dr. Govindarajan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche . Dr. Govindarajan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sarepta. Dr. Govindarajan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amicus. Dr. Govindarajan 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. Govindarajan has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion. Dr. Govindarajan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for MT pharma . Dr. Govindarajan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Catalyst. Dr. Govindarajan has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Argenx. Dr. Govindarajan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Biohaven. Dr. Govindarajan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. Dr. Govindarajan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Takeda. Dr. Govindarajan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Abbvie. The institution of Dr. Govindarajan has received research support from Band of Hope . The institution of Dr. Govindarajan has received research support from Alexion. Dr. Govindarajan has received publishing royalties from a publication relating to health care.
Kateryna Kurako, MD	Dr. Kurako has nothing to disclose.

��ɫ��

��ɫ��