2026 American Academy of Neurology Abstract Website

Objective:

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

Background:

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.

Design/Methods:

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.

Results:

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.

Conclusions:

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.