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Abstract Details

Longitudinal Cognitive Assessment in Stiff Person Syndrome: A Case Report
Autoimmune Neurology
P5 - Poster Session 5 (11:45 AM-12:45 PM)
2-001

To characterize longitudinal changes in cognitive and psychological symptoms in a 54-year-old woman with stiff person syndrome (SPS).

Cognitive symptoms are commonly reported among individuals with SPS; however, etiology of cognitive impairment in SPS is multi-factorial, as cognitive and psychological symptoms often co-occur. In a recent retrospective study of 20 SPS patients, prevalence of depression (80%) and anxiety (35%) symptoms was greater than that of the general population, and over 40% demonstrated cognitive deficits in verbal learning and recall memory, verbal fluency, processing speed, and/or attention. Thus, cognitive impairments may reflect a SPS neurodegenerative process and/or interaction of psychological and cognitive factors.

Two cognitive assessments were performed in a woman with SPS in 2020 and 2025. Standardized measures included: Test of Premorbid Function, Judgement of Line Orientation, Boston Naming Test, California Verbal Learning Test (CVLT-3), Brief Visuospatial Memory Test, Symbol Digit Modalities Test, Digit Span, Controlled Oral Word Association Test, and Animal Naming Test. The PHQ-9 and GAD-7 were also administered.
A 54-year-old woman with classic SPS and ongoing cognitive concerns underwent neurocognitive testing. Her SPS diagnosis preceded her first assessment by nine years, and her Modified Rankin Scale was 2 at initial testing, followed by 3 on follow-up testing. Medications – immunoglobulin, clonazepam, and pregabalin – remained stable between assessments. Over the 5 years between assessments, the patient demonstrated clinically significant declines (>1 SD change) on measures of working memory (Digit Span) and verbal learning efficiency (CVLT-3). Other domains were stable. Across both assessments, the patient demonstrated consistent PHQ-9 and GAD-7 scores, reporting moderate levels of depression and anxiety.

Findings indicate some areas of cognitive decline may be associated with SPS, including when symptoms co-occur with persistent psychological symptoms. Results underscore the importance of longitudinal cognitive assessment in SPS to inform potential cognitive rehabilitation and mental health interventions.
Authors/Disclosures
Abbey J. Hughes, PhD (Johns Hopkins University School of Medicine)
PRESENTER 		The institution of Dr. Hughes has received research support from Consortium of MS Centers. The institution of Dr. Hughes has received research support from National MS Society. The institution of Dr. Hughes has received research support from Department of Defense. Dr. Hughes has received personal compensation in the range of $0-$499 for serving as a Consultant/Speaker with Can Do Multiple Sclerosis.
Sarah Snoops, RN Mrs. Snoops 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.