Abstract Details

Title Does Age Matter? Clinical Characteristics of Stiff Person Syndrome Spectrum Diagnosed After Age 50

Topic Autoimmune Neurology

Presentation(s) P4 - Poster Session 4 (8:00 AM-9:00 AM)

Poster/Presentation
Number 2-006

Objective Characterize late-onset stiff person syndrome spectrum disorders (LO-SPSD).

Background Most patients with SPSD experience symptom onset younger than age 50, but some develop symptoms later in life. Limited data exist regarding the clinical features and disability in LO-SPSD.

Design/Methods An observational study of SPSD patients seen in a tertiary referral clinic from 1997 to 2025 was performed. Demographics, clinical features, time to diagnosis and ambulation measures were collected.

Results There were 90 patients with symptom onset >50-years-old. Most were female (76%), and white was the most common race (70%) followed by Black (22%). Mean age at diagnosis was 60.2 (SD=7.5), with a median time to diagnosis of 16.8 (IQR=8.2-29.6) months versus 48.6 (IQR=19.4-91.9) months for <50-years-old. Most patients were anti-GAD65-antibody positive (88%). Over half had a classic SPS phenotype (58%), followed by SPS-plus (21%). Misdiagnosis rate was 51% (versus 67% in those <50-years-old), with the most common initial incorrect diagnoses being vestibular dysfunction (27%), cervical spondylosis (20%), or dystonia (13%). The most common initial symptoms were stiffness (50%), spasms (28%) and cerebellar/brainstem dysfunction (19%). At their first evaluation most (88%) patients >50-years-old reported gait difficulties and 60% needed a walking aid. The need for gait assistive device remained relatively stable over time, with 55% requiring one at last follow-up (compared to 37.5% at first visit and 36% at last visit in the <50-years-old group).

Conclusions LO-SPSD had lower rates of misdiagnosis and shorter time to diagnosis than those under 50, however older patients had more gait difficulty, but this remained stable over time. Monitoring and counseling around ambulation safety is particularly important in an aging SPSD population.

Authors/Disclosures
Clare M. Lambert, MD PRESENTER	Dr. Lambert has nothing to disclose.
Hanyeh Afshar	Ms. Afshar has nothing to disclose.
Asli Buyukkurt, MD	Dr. Buyukkurt has nothing to disclose.
Daniela Riveros Acosta, MD	Dr. Riveros Acosta has nothing to disclose.
Martin M. O'Donnell, MB BCh BAO (HSE Ireland / Beaumont Hospital)	Dr. O'Donnell has nothing to disclose.
Sarah Snoops, RN	Mrs. Snoops has nothing to disclose.
Barrett Crawford	Mr. Crawford has nothing to disclose.
Scott D. Newsome, DO, FAAN (Johns Hopkins Hospital)	Dr. Newsome has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. The institution of Dr. Newsome has received research support from Biogen. The institution of Dr. Newsome has received research support from Genentech/Roche. The institution of Dr. Newsome has received research support from Department of Defense. The institution of Dr. Newsome has received research support from Patient Centered Outcomes Research Institute. The institution of Dr. Newsome has received research support from National MS Society. The institution of Dr. Newsome has received research support from Lundbeck. The institution of Dr. Newsome has received research support from Sanofi. The institution of Dr. Newsome has received research support from Kyverna Therapeutics. Dr. Newsome has received personal compensation in the range of $10,000-$49,999 for serving as a Lead PI for Clinical Trial with Roche.

��ɫ��

��ɫ��