Abstract Details

Title Refractory Myasthenia Gravis Exacerbation associated with Marginal Zone Lymphoma

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P7 - Poster Session 7 (8:00 AM-9:00 AM)

Poster/Presentation
Number 11-009

Objective To demonstrate a case of Myasthenia Gravis(MG) with an exacerbation refractory to conventional treatment associated with Marginal Zone Lymphoma.

Background Myasthenia Gravis is an autoantibody mediated cause for neuromuscular weakness which may be a paraneoplastic manifestation of an underlying tumor. It is most commonly associated with Thymoma but has been described in the literature to present in association with some B cell lymphomas.

Design/Methods A 73 year old man with an untreated low grade marginal zone lymphoma (diagnosed 1 year 9months prior), received a recent diagnosed of seronegative MG (negative AChR, MuSK, LRP, VGCC antibodies) 6 months prior to presentation (and was kept on maintenance steroids), presented to the hospital with worsening ptosis, dysphagia, diplopia, and proximal muscle weakness triggered by shingles. He was admitted to the stepdown unit, treated with a 5-day- course of 2g/kg IVIg, high dose steroids, and Acyclovir. He worsened and was intubated 5 days after completing IVIg. Neuroaxis imaging was negative. Lumbar Puncture was obtained and significant for mildly elevated protein but negative cells, cytology, cytometry, infectious work up and autoimmune/paraneoplastic antibodies. Systemic imaging revealed worsening retroperitoneal and mesenteric adenopathy and persistent splenomegaly. With a refractory course and failed extubation, the patient was started on Rituximab 375mg/m2 weekly for 4 weeks (lymphoma dosing) which after the patient was weaned off the ventilator and discharged with improvement in weakness 2 months after admission.

Results -

Conclusions MG is an autoantibody mediated disease which may be a paraneoplastic manifestation of an underlying neoplasm, most commonly a thymoma. Refractory MG exacerbation may respond to treatment of the underlying neoplasm.

Authors/Disclosures
Karan Philip, MBBS PRESENTER	Dr. Philip has nothing to disclose.
Christina Xia, MD	Dr. Xia has nothing to disclose.
Ruham Alshiekh Nasany, MD	Dr. Alshiekh Nasany has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for J&J.
Jenny Meyer, MD (Upstate Medical University)	The institution of Dr. Meyer has received research support from Grifols. The institution of Dr. Meyer has received research support from Medicinova.

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