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Abstract Details

Myelopathy in the Setting of Malignancy: The Importance of a Thoughtful Approach to Autoantibody Testing Based on Clinical Presentation
Autoimmune Neurology
Autoimmune Neurology Posters (7:00 AM-5:00 PM)
044
To describe two cases of paraneoplastic autoimmune CNS disease with emphasis on the importance of autoantibody testing directed by the clinical phenotype.
Neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein associated disease (MOGAD) are most frequently non-paraneoplastic, autoimmune conditions but have been described in a paraneoplastic context.  NMOSD is characterized by inflammatory attacks to the optic nerves and spinal cord and is associated with an aquaporin 4 (AQP4)-immunoglobulin G-mediated astrocytopathy. MOGAD has a wider clinical spectrum and is a pathophysiologically distinct oligodendrogliopathy.
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Case 1:

A 73-year-old male presenting with subacute-onset bilateral lower extremity paraplegia with a history significant for lung adenocarcinoma treated previously with pembrolizumab. Neural autoantibody testing of both CSF and serum via a comprehensive encephalopathy/paraneoplastic evaluation was unrevealing. CSF showed elevated protein, and serum was notable for AQP4-IgG positivity at 1:80 on cell-based assay. He was treated with methylprednisolone, IVIg, rituximab, and plasmapheresis, without significant neurologic recovery.

Case 2:

A 64-year-old female presenting with subacute progression of weakness, vision changes, and confusion for two months.  MRIs showed T2 hyperintense lesions supratentorially and short-segment lesions in the cervical and thoracic spinal cord. CT thorax showed a right lower lobe mass, consistent with poorly-differentiated lung carcinoma on biopsy. Neural autoantibody testing of both CSF and serum via a comprehensive encephalopathy/paraneoplastic evaluation was unrevealing. CSF was significant for unique oligoclonal bands. Serum MOG IgG was elevated to 1:160. She was treated with methylprednisolone and plasmapheresis with mild improvement of weakness and cognition.
NMOSD and MOGAD are frequently autoimmune conditions but have been described in a paraneoplastic context. Most available testing evaluations/panels designed to query a paraneoplastic etiology do not include AQP4 or MOG IgG, and thus a thoughtful approach to testing based on the clinical phenotype is essential whether the suspected etiology is autoimmune, cancer immunotherapy-related, or paraneoplastic. 
Authors/Disclosures
Stefanie J. Rodenbeck, MD (Indiana University)
PRESENTER 		Dr. Rodenbeck has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion Pharmaceuticals. Dr. Rodenbeck has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. Dr. Rodenbeck has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen.
Paul D. Crane, MD (University of Colorado) Dr. Crane has nothing to disclose.
Adam F. Sitzmann, MD Dr. Sitzmann has nothing to disclose.
Stacey Clardy, MD, PhD, FAAN (University of Utah) Dr. Clardy has received personal compensation for serving as an employee of Veterans Health Administration (VHA). Dr. Clardy has received personal compensation for serving as an employee of University of Utah Health. Dr. Clardy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AstraZeneca/Alexion. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen/Horizon. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arialys. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. Dr. Clardy has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology/AAN Publications. The institution of Dr. Clardy has received research support from NIH/NINDS. The institution of Dr. Clardy has received research support from SRNA. The institution of Dr. Clardy has received research support from Alexion/AstraZeneca. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a AAN Summer Meeting CoDirector Travel and Lodging with AAN. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel/Lodging/Honoraria with U of Iowa, Miami, Stanford, Barrow, Beaumont Health, CCF, Emory, Penn State, Mayo Clinic, Walter Reed.