Abstract Details

Title Isolated Progressive Myelopathy Presenting as Initial Symptom in a Patient with Nicotinic-Ganglionic Acetylecholine Receptor Autoimmunity

Topic Neuro-oncology

Presentation(s) P07 - (-)

Poster/Presentation
Number 004

Objective

Background BACKGROUND: The Alpha3-AChR autoantibody has been linked to diverse neurological symptoms in the setting of paraneoplastic disease. The autoantibody has a strong association with cancer, most commonly adenocarcinoma. We present the case of a young gentleman who developed isolated myelopathy as the initial and predominant manifestation of Alpha3-AChR autoantibody autoimmunity. To the best of our knowledge, this is the first case described in the literature with these symptoms associated with this autoantibody.

Design/Methods DESIGN/METHODS: A 50-year-old male with a history of dermatomyositis currently on remission, discoid lupus and gastroparesis presents with insidious onset, progressive gait difficulties for approximately 2 years. His neurological examination demonstrated upper motor neuron signs in lower extremities with a spastic gait. Extensive evaluation for causes of myelopathy was negative. An extensive paraneoplastic panel was negative except for positive Alpha3-AChR autoantibody.

Results RESULTS: Serologic testing revealed moderate values of Alpha3-AChR autoantibody. Additional testing for occult neoplasm showed axillary, celiac and gastrohepatic lymphadenopathy suspicious for malignancy. The diagnosis of gastric adenocarcinoma was later confirmed. Approximately 1 year after the onset of his neurological symptoms, he developed weight loss, fatigue, cognitive decline and mild autonomic symptoms. A trial of intravenous immunoglobulin (IVIG) resulted in partial and transient improvement in his gait and cognitive difficulties.

Conclusions CONCLUSIONS: The spectrum of neurological manifestations associated with Alpha3-AChR autoantibody appears to be much broader than originally recognized. Pandysautonimia can be mild, and both central and peripheral nervous systems might be affected. The detection of this antibody must prompt consideration of an underlying malignancy. Identification and treatment of the primary tumor is critical. The goal of immunotherapy is to halt neurologic symptom progression, preserve stability and attempt reversibility. However, the prognosis in chronic paraneoplastic myelopathies remains poor.

Authors/Disclosures
Fumin Tong, MD, PhD (Neurology Group of Bergen County) PRESENTER	No disclosure on file
Adolfo Ramirez Zamora, MD (University of Louisville)	Dr. Ramirez Zamora has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Medtronic. Dr. Ramirez Zamora has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cerevel therapeutics. Dr. Ramirez Zamora has received personal compensation in the range of $0-$499 for serving as a Consultant for NeuroPacs. Dr. Ramirez Zamora has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Boston Scientific. Dr. Ramirez Zamora has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Iota Inc. Dr. Ramirez Zamora has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Maplight. Dr. Ramirez Zamora has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for NIH. The institution of Dr. Ramirez Zamora has received research support from Parkinsons Foundation.
Xavier Montalban, MD, PhD, FAAN (Vall Hebron University Hospital-Multiple Sclerosis Centre of Catalonia)	The institution of Dr. Montalban has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Biogen. The institution of Dr. Montalban has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Merck/ EMD Serono. The institution of Dr. Montalban has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Novartis. The institution of Dr. Montalban has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Teva. The institution of Dr. Montalban has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Hoffmann-La Roche. The institution of Dr. Montalban has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Actelion/ Janssen Pharmaceuticals. The institution of Dr. Montalban has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for BMS/ Celgene. The institution of Dr. Montalban has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi/ Genzyme. The institution of Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BIAL PD. The institution of Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving as a Consultant for PeerVoice. The institution of Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Samsung Biosys. The institution of Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Zenas BioPharma. Dr. Montalban has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Actelion/ Janssen Pharmaceuticals. Dr. Montalban has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Montalban has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for BMS/ Celgene. Dr. Montalban has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck/ EMD Serono. Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Hoffmann-La Roche. Dr. Montalban has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi-Genzyme. The institution of Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Immunic Therapeutics. The institution of Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Viatris/ Mylan. The institution of Dr. Montalban has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sandoz. The institution of Dr. Montalban has received research support from Biogen. The institution of Dr. Montalban has received research support from Hoffmann-La Roche. The institution of Dr. Montalban has received research support from Sanofi/ Genzyme. The institution of Dr. Montalban has received research support from Merck/ EMD Serono. The institution of Dr. Montalban has received research support from Novartis. The institution of Dr. Montalban has received research support from Teva Pharmaceuticals. The institution of Dr. Montalban has received research support from Actelion/ Janssen Pharmaceuticals. The institution of Dr. Montalban has received research support from BMS/ Celgene.

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