Neurology. 2025 Apr 8;104(7_Supplement_1):4269. doi: 10.1212/WNL.0000000000211560. Epub 2025 Apr 7.
ABSTRACT
OBJECTIVE: Objectives of this study are to retrospectively analyze Healthcare Resource Utilization (HCRU) and associated costs in 59 patients diagnosed with SPSD at a large academic center to identify areas for interventions.
BACKGROUND: Stiff Person Syndrome Spectrum Disorder (SPSD) is a rare autoimmune disorder characterized by progressive muscle stiffness and painful spasms.
DESIGN/METHODS: We queried Health Data Compass (HDC), an electronic health data repository for a large academic healthcare system, from 2012 through 2022 for all patients ≥ 18-year-old with ICD-10 codes pertaining to SPSD in a recent epidemiology study to define incidence and prevalence of SPSD (Neurology, in press). In this current study, we then used the HDC database in this well-defined SPSD cohort to capture electronic health records from a large academic health system and claims data from the Colorado All Payers Claim Database to further define HCRU in this population.
RESULTS: 273 patients met the initial inclusion criteria using ICD-10 codes; 59 were confirmed to have SPSD via chart review. Based on our cohort of 59 patients, the mean age of patients was 49.7 years (SD=12.9), 59.3% were female, and 59.3% were antibody-positive. 81.4% self-identified as White, 8.5% Black, 1.2% American Indian or Alaskan Native, and 6.8 % other. 91.5% self-reported as non-Hispanic. 72.9% had ≥ 1 electromyography/nerve conduction study and 81.3% had ≥ 1 brain magnetic resonance image in our system. Additional data collected includes hospitalizations, emergency room and outpatient visits, laboratory and radiology services, and pharmacy related services to evaluate all-cause and SPSD related HCRU and costs.
CONCLUSIONS: Using a well-defined, population-based cohort, we can explore HCRU patterns for patients with SPSD. Understanding the true disease burden of SPSD is fundamental to ensuring optimal patient care, allocating appropriate system resources, and defining standards of care for this rare disease. Disclaimer: Abstracts were not reviewed by Neurology® and do not reflect the views of Neurology® editors or staff. Disclosure: Dr. Lowe has nothing to disclose. Mr. Gutierrez has nothing to disclose. Dr. Barlev has received personal compensation for serving as an employee of Kyverna. Dr. Crane has nothing to disclose. Mr. Engebretson has nothing to disclose. Stefan Sillau has nothing to disclose. 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 $5,000-$9,999 for serving as a Consultant for MJH Lifesciences. Dr. Nair has received personal compensation in the range of $5,000-$9,999 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 EMD Serono. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amgen. 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 as a Consultant for Applied Patient Experience. Dr. Nair has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Sanofi-Genzyme. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amgen. Dr. Nair has received personal compensation in the range of $500-$4,999 for serving as an officer or member of the Board of Directors for ICER. 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. The institution of Dr. Nair has received research support from Sanofi Genzyme. The institution of Dr. Nair has received research support from Kyverna Therapeutcis. Dr. Nair has a non-compensated relationship as a Board Member with Center for Improving Value in Healthcare that is relevant to AAN interests or activities. Dr. Carlson has received research support from Horizon Therapeutics (Amgen). 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 $10,000-$49,999 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. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Kyverna . 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. The institution of Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. 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 Horizon/Amgen. 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. The institution of Dr. Piquet has received research support from Kyverna . 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.
PMID:40194238 | DOI:10.1212/WNL.0000000000211560