Abstract Details

Title A case of primary CNS lymphoma associated with neuromyelitis optica spectrum disorder: Is there a paraneoplastic link?

Topic Multiple Sclerosis

Presentation(s) P1 - Poster Session 1 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-078

Objective We report a unique case of neuromyelitis optica spectrum disorder (NMOSD) in a patient with concurrent primary CNS lymphoma (PCNSL) to highlight the importance of considering underlying malignancy in aquaporin-4 antibodies (APQ4-ab) seropositive NMOSD, specifically in those with atypical presentations and/or poor response to anti-inflammatory and disease-modifying therapies.

Background NMOSD has been previously associated with non-CNS malignancies suggesting that in some cases NMOSD may be a paraneoplastic condition. In addition, it has been previously reported that a subset of patients who were eventually diagnosed with PCNSL have presented with “sentinel” lesions resembling NMOSD or other demyelinating disorders. However, NMOSD has not been linked with any primary CNS malignancy. Here, we describe a unique case of AQP4-seropositive NMOSD associated with PCNSL.

Design/Methods NA

Results CASE REPORT: A 51-year-old woman presented to our university hospital with subacute bilateral upper extremity weakness. Brain magnetic resonance imaging (MRI) demonstrated a longitudinally-extensive contrast-enhancing lesion in the cervical spine as well as multiple large FLAIR T2 hyperintense brain lesions. She tested positive for APQ4-ab, prompting the diagnosis of APQ4-ab seropositive NMOSD. Although the spinal cord lesion responded well to steroids and rituximab treatment, she returned several months later with dysphagia, dysarthria, and bilateral lower extremity weakness. She transiently improved with high-dose steroids, however, relapsed again thereafter. Repeat imaging demonstrated a resolution of the initial cervical lesion but portrayed a worsening of the left frontal lesion and contrast-enhancing corpus callosum lesion. A stereotactic-guided biopsy was subsequently performed and neuropathological results returned positive for large B-cell PCNSL. Symptoms, imaging findings and AQP4-ab titer improved with treatment of PCNSL.

Conclusions We are reporting a rare case of AQP4-ab seropositive NMOSD associated with PCNSL. This raises the concern of NMOSD as a paraneoplastic condition. Hence, atypical cases may require additional work up to rule out malignancies including primary CNS tumors.

Authors/Disclosures
Emilio R. Garrido Sanabria, MD, PhD (Physicians Regional Medical Center) PRESENTER	Dr. Garrido Sanabria has nothing to disclose.
Ryan K. Jones, MD	No disclosure on file
Alexander C. Ou, MD (Kelsey-Seybold Clinic)	Dr. Ou has nothing to disclose.
Olga Syritsyna, MD	Dr. Syritsyna has nothing to disclose.
	No disclosure on file
	No disclosure on file
Agnes Kowalska, MD (SUNY At Stony Brook)	Dr. Kowalska has nothing to disclose.
	No disclosure on file
Patricia K. Coyle, MD, FAAN (SUNY At Stony Brook)	Dr. Coyle has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Accordant. Dr. Coyle has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amgen. Dr. Coyle has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Sanofi Genzyme. Dr. Coyle has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Novartis. Dr. Coyle has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for GlaxoSmithKline. Dr. Coyle has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Horizon Therapeutics. The institution of Dr. Coyle has received research support from CorEvitas LLC. The institution of Dr. Coyle has received research support from Genentech/Roche. The institution of Dr. Coyle has received research support from NINDS. The institution of Dr. Coyle has received research support from Sanofi Genzyme. The institution of Dr. Coyle has received research support from Cleveland Clinic.

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