Therapeutic Dilemma in Generalized Myasthenia Gravis and Good's Syndrome Overlap (P02.195)

OBJECTIVE: To report the clinical presentation and treatment options in a 65 year old male with myasthenia gravis and Good9s syndrome overlap. BACKGROUND: Good9s syndrome (thymoma with immunodeficiency) is a rare cause of combined B and T cell immunodeficiency in adults.Good9s syndrome can be uncommonly associated with myasthenia gravis (MG).Immunosuppressive therapy for MG in patients with Good9s syndrome is complicated by development of recurrent opportunistic infections. DESIGN/METHODS: Case Report. 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 Good9s syndrome. CONCLUSIONS: Recurrent opportunistic infections in the setting myasthenia (with or without immunosuppressive therapy) and thymoma should raise the suspicion of Good9s syndrome. Periodic long term intravenous immunoglobulin can be a treatment option for both MG and Good9s syndrome. Disclosure: Dr. Salanga has nothing to disclose. Dr. Govindarajan has nothing to disclose. Dr. Kurako has nothing to disclose.