Abstract Details

Title Paraneoplastic Neurologic Syndromes Associated with Lymphomas

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (12:00 PM-1:00 PM)

Poster/Presentation
Number 061

Objective

Describe paraneoplastic neurologic syndromes (PNS) related to an underlying lymphoma.

Background Although neurologic symptoms in patients with lymphomas are often secondary to direct tumor involvement or chemotherapy, PNS can occur in a minority and needs to be better characterized.

Design/Methods We retrospectively identified lymphoma associated PNS patients evaluated at our institution (2005-2020).

Results From our Mayo Clinic PNS cohort (n=212), we identified 10 patients with PNS associated with lymphoma (5%), of whom five (50%) were females. Median age of PNS onset was 48 years (range 25-77). Eight (80%) patients had a neuromuscular presentation with polyradiculoneuropathy (n=6, 60%) being the most common phenotype; two (20%) patients had a rapidly progressive cerebellar syndrome. Other presentations included recurrent plexopathy (n=1) and cranial neuropathy with dysautonomia (n=1). Malignancy associations included Hodgkin’s lymphoma in 5 (50%) patients, while others were non-Hodgkin lymphomas that included mantle cell (n=1), marginal zone (n=1), mucosa associated lymphoid tissue (MALT) (n=1), diffuse large B-cell (n=1) and lymphoplasmacytic lymphoma (n=1). Only three patients (30%) had a high-risk autoantibody associated with the PNS, and all were Purkinje cell cytoplasmic antibody type Tr (PCA-Tr). All patients received cancer treatment while immunotherapy was administered in 50% patients. Nine patients achieved hematologic remission, 6 of whom demonstrated improvement or stabilization of the PNS. One patient who had an initial neurological improvement deteriorated later and died of cancer recurrence. Both patients with rapidly progressive cerebellar syndrome showed no improvement. At last follow up, only 50% of patients had mRS≤3.

Conclusions In our study polyradiculoneuropathy was the most common presenting PNS phenotype associated with lymphoma. High-risk paraneoplastic antibody (PCA-Tr IgG) association was limited to ataxia/cranial neuropathy phenotype. Most patients improved from hematological perspective (60%), however, even after hematologic remission, debilitating neurologic symptoms often persisted.

Authors/Disclosures
Hannah Zhao-Fleming, MD, PhD PRESENTER	Dr. Zhao-Fleming has nothing to disclose.
Naveen K. Paramasivan, MD (Flat B2, AKB SPRINGS)	Dr. Paramasivan has nothing to disclose.
Anastasia Zekeridou, MD, PhD, FAAN (Neuroimmunology Laboratory, Mayo Clinic)	The institution of Dr. Zekeridou has received research support from Roche/Genentech. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care. Dr. Zekeridou has received intellectual property interests from a discovery or technology relating to health care.
Andrew McKeon, MD (Mayo Clinic)	The institution of Dr. McKeon has received research support from National Institutes of Health. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received publishing royalties from a publication relating to health care.
Sean J. Pittock, MD, FAAN (Mayo Clinic Dept of Neurology)	Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche/Genentech. The institution of Dr. Pittock has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion/AstraZeneka. The institution of Dr. Pittock has received research support from NIH. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received publishing royalties from a publication relating to health care.
Divyanshu Dubey, MD, FAAN (Mayo Clinic)	The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from UCB. Dr. Dubey has received research support from David J. Tomassoni ALS Research Grant Program . Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care.

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