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Abstract Details

A Single-center Case Series of Patients with Stiff-person Syndrome
Autoimmune Neurology
P1 - Poster Session 1 (8:00 AM-9:00 AM)
14-001

SPS is a rare autoimmune disorder characterized by progressive muscle stiffness and painful spasms estimated to occur in 1-2 per million people within the United States, although large, population-based epidemiological studies are lacking. There are no formal consensus criteria for SPS. Here, we describe the epidemiology and clinical presentation of SPS in a large US health system utilizing a unique, linked data resource. 

Describe the epidemiology and patient characteristics of stiff person syndrome (SPS) patients seen at a large academic health system. 

We queried Health Data Compass (HDC), a linked dataset connecting electronic health records from a single academic health care system with the Colorado All Payers Claim Database from 2012 through 2022 for all patients ≥ 18-year-old with ICD-10 codes pertaining to Stiff Person Syndrome or Stiff-Man Syndrome [G25.82] with subsequent record review for diagnostic confirmation. The SPS diagnosis will be adjudicated and proposed diagnostic criteria will be applied to understand the sensitivity and specificity of this criteria in our cohort.

261 patients met the initial inclusion criteria. The database population over the interval was 2,802,246 persons. The prevalence for SPS was 9.3 per 100,000 persons (95% CI:(8.2, 10.4)). The mean age of patients was 51.8 years (SD=15.7), 57.5% were female, and 82.4% self-identified as Caucasian, 7.6% Black, and 10 % other. Based on our HDC dataset, 34 patients had high (> 20 nmol/L or >250 IU/mL) GAD65 antibody titers and 3 were glycine antibody positive. 5.75% of patients were on IVIG and 16.86% on rituximab. Additional record review is ongoing to confirm serostatus, evaluate immunotherapy, and characterize clinical presentation and medical comorbidities.

Understanding the true disease burden and onset of SPS is fundamental to ensuring optimal patient care. Characterizing SPS will provide an opportunity to ensure appropriate allocation of system resources and to improve upon current treatment standards.

Authors/Disclosures
Paul D. Crane, MD (University of Colorado)
PRESENTER
Dr. Crane has nothing to disclose.
Russell J. Van Coevering III, MD (Renown Medical Center) Dr. Van Coevering has nothing to disclose.
Stefan Sillau No disclosure on file
Eric Engebretson (University of Colorado Denver) Mr. Engebretson has nothing to disclose.
Brooke Valdez (University of Colorado) Brooke Valdez has nothing to disclose.
Kavita Nair, PhD, FAAN (University of Colorado) Dr. Nair has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Bristol Meyers Squibb. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Nair has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for PhRMA Foundation. Dr. Nair has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Genentech. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for MJH Healthcare. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD Serono. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Horizon. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Anylam . Dr. Nair has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sanofi-Genzyme. The institution of Dr. Nair has received research support from Genentech. The institution of Dr. Nair has received research support from Novartis. The institution of Dr. Nair has received research support from Genentech. The institution of Dr. Nair has received research support from Phrma Foundation. The institution of Dr. Nair has received research support from Bristol Meyers Squibb. The institution of Dr. Nair has received research support from Novartis. The institution of Dr. Nair has received research support from NINDS.
Aaron M. Carlson, MD (University of Colorado, School of Medicine, Department of Neurology) Dr. Carlson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Medscape. Dr. Carlson has received research support from Horizon Therapeutics. Dr. Carlson has a non-compensated relationship as a Health Service Subcommittee Member with American Academy of Neurology that is relevant to AAN interests or activities.
Amanda L. Piquet, MD, FAAN (University of Colorado) The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion. The institution of Dr. Piquet has received personal compensation in the range of $0-$499 for serving as a Consultant for UCB. The institution of Dr. Piquet has received personal compensation in the range of $0-$499 for serving as a Consultant for EMD Serono. Dr. Piquet 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. Piquet has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. The institution of Dr. Piquet 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. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Sands Anderson PC. Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Joe Jones Law Firm. The institution of Dr. Piquet has received research support from Rocky Mountain MS Center. The institution of Dr. Piquet has received research support from Novartis. The institution of Dr. Piquet has received research support from Abbvie. The institution of Dr. Piquet has received research support from Roche/Genentech. The institution of Dr. Piquet has received research support from NYU. The institution of Dr. Piquet has received research support from Anokion. The institution of Dr. Piquet has received research support from UCB . The institution of Dr. Piquet has received research support from Foundation for Sarcoidosis. Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as a Litigative Consultant with US-Dept HHS/DICP. Dr. Piquet has a non-compensated relationship as a Medical Advisory Board Member with Autoimmune Encephalitis Alliance (AEA) that is relevant to AAN interests or activities. Dr. Piquet has a non-compensated relationship as a Medical Advisory Board Member with Stiff Person Syndrome Research Foundation (SPSRF) that is relevant to AAN interests or activities.