Abstract Details

Title Acute Autonomic Symptoms with Anti-MAG Neuropathy as a Presentation of Small B Cell Lymphoma

Topic Autonomic Disorders

Presentation(s) P03 - (-)

Poster/Presentation
Number 023

Objective

Background BACKGROUND: MAN classically presents with distal symmetric sensorimotor polyneuropathy. Autonomic symptoms are uncommon with MAN. Few cases have reported a small B cell lymphoma presenting with MAN associated autonomic symptoms.

Design/Methods DESIGN/METHODS: Case Report.

Results RESULTS: 68 year old lady presented with progressive ascending numbness, weakness of her extremities and balance problems. On exam she was diffusely weak (MRC grading 4/5), had reduced perception to all modalities of sensation with marked impairment of vibration and position sense in lower limbs. Reflexes were diminished throughout and Romberg was positive. Initial lab work including TSH, Vitamin B12, Hb1c, routine labs was normal. Patient then developed acute urinary retention; severe orthostatic hypotension and symptomatic bradycardia so much so that she needed temporary pacing. Work up during this time revealed M spike on serum electrophoresis with IgM kappa on immunofixation. IgM titers were 568mg/dl (normal 40 - 230 mg/dl). Initially she was thought to have MGUS related neuropathy but further work up showed anti MAG antibody titers >1:102400 (normal<1:1600). Bone marrow biopsy showed small atypical lymphoid cells which stained positive for CD20, PAX-5, with rare CD138 positive plasma cells consistent with a small B-cell lymphoproliferative disorder. She is currently being treated with Rituximab with significant improvement in her neuropathic symptoms.

Conclusions CONCLUSIONS: Acute autonomic symptoms can be a rare and a confusing clinical manifestation of anti-MAG distal symmetric polyneuropathy.Thorough work-up including bone marrow biopsy is needed to rule out underlying B cell lymphoma in the presence of a monoclonal gammopathy.

Authors/Disclosures
Dennys Reyes, MD (Reyes Neurology) PRESENTER	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.
Efrain D. Salgado, MD (Cleveland Clinic Florida)	Dr. Salgado has nothing to disclose.
Per Soelberg Sorensen	No disclosure on file

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