Abstract Details

Title Population-Based Epidemiology Study of Paraneoplastic Neurological Syndromes

Topic Autoimmune Neurology

Presentation(s) S7 - Autoimmune and Paraneoplastic Neurologic Disorders: Diagnosis, Epidemiology, and Pathophysiology (2:00 PM-2:08 PM)

Poster/Presentation
Number 001

Objective To evaluate the incidence, prevalence, and associated morbidity of paraneoplastic neurological syndromes (PNS).

Background Population-based epidemiological data for PNS in the United States (US) is lacking.

Design/Methods Patients meeting 2004 “definite” PNS diagnostic criteria or paraneoplastic myasthenia gravis in Olmsted County, Minnesota, diagnosed between 1/1/1987 and 12/31/2018, were identified using the medical records-linkage system of the Rochester Epidemiology Project (REP). Age/sex-specific population counts were obtained from REP resources for 1/1/2014 (prevalence denominator) and annually for 1987-2018 (incidence denominator). Incidence/prevalence rates were directly adjusted to the age/sex-distribution of the 2010 total US population. Morbidity was estimated using disability-adjusted life years (DALYs; years lost to disability [YLD] plus years of life lost [YLL]).

Results Of the 28 PNS patients identified (n=14, 50% female) the median age at diagnosis was 54.5 (IQR 46.5-69) years. There were 6 incident cases from 1/1/1987-12/31/2002, 19 incident cases from 1/1/2003-12/31/2018, and 8 prevalent cases on 1/1/2014. PNS prevalence was 5.39/100,000 people. Incidence from 1987-2018 was 0.63/100,000 person-years. PNS incidence increased over time from 0.37/100,000 person-years (1987-2002) to 0.82/100,000 person-years (2003-2018) (p=0.06). Cancer diagnosis were thymoma/thymic carcinoma (n=6, 21%), breast adenocarcinoma (n=4, 14%), seminoma (n=4 14%), small cell lung cancer (n=4, 14 %), non-small cell lung cancer (n=2, 7%), lymphoma (n=2, 7%), and others (n=6, 21%). Eighteen (64%) patients were antibody positive: acetylcholine-binding receptor (n=6), KLHL11 (n=3), PCA1 (n=1), ANNA1 (n=1), ANNA2 (n=1), CRMP5 (n=1), LGI1 (n=1), AMPA-R (n=1), neuronal intermediate filament (n=1) and unclassified neural autoantibody (n=2). At last follow-up, 8/28 patients (29%) required a gait aid, and by the end of the study period 15 (54%) had died. DALYs over the study period for 28 PNS patients were 518.2 years (72.9 YLD, 445.3 YLL).

Conclusions This study provides the first US based PNS incidence and prevalence and highlights the high morbidity and mortality.

Authors/Disclosures
Shailee S. Shah, MD (Vanderbilt Multiple Sclerosis Center) PRESENTER	Dr. Shah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. Dr. Shah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Shah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Shah 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. Shah has received publishing royalties from a publication relating to health care.
Eoin P. Flanagan, MBBCh, FAAN (Mayo Clinic)	The institution of Dr. Flanagan 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. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. The institution of Dr. Flanagan has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Pharmacy times. The institution of Dr. Flanagan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. The institution of Dr. Flanagan has received research support from Viela Bio. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Roche. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has a non-compensated relationship as a Member of medical Advisory Board with The MOG Project that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Journal of The Neurologic Sciences that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Neuroimmunology Reports that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology, Neuroimmunology Neuroinflammation (N2) Journal that is relevant to AAN interests or activities.
Pritikanta Paul, MD (University of California, San Francisco)	The institution of Dr. Paul has received research support from ZS Associates.
	No disclosure on file
	No disclosure on file
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 UCB. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys Therapeutics. 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. 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 Arialys. 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. The institution of Dr. Pittock has received research support from Alexion/AstraZeneka. The institution of Dr. Pittock has received research support from F. Hoffman/LaRoche/Genentech. 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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