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Abstract Details

Neuropsychological performance and psychiatric symptoms in stiff person syndrome
Multiple Sclerosis
MS and CNS Inflammatory Disease Posters (7:00 AM-5:00 PM)
187
To examine the pattern of cognitive deficits and psychiatric symptoms in patients with stiff person syndrome (SPS).

SPS is an immune-mediated neurological disorder that presents with a spectrum of symptoms and is most commonly associated with anti-glutamic acid decarboxylase (anti-GAD) antibodies. SPS has been associated with lower than expected performance on cognitive testing relative to estimated premorbid intelligence.  Additionally, the presence of anti-GAD antibodies has been associated with an elevated risk of cognitive impairment in patients with diabetes and in animal models.

A retrospective review of medical records from 1997 to 2020 at Johns Hopkins Hospital was performed. SPS patients who had received neuropsychological testing as part of routine clinical care for subjective cognitive complaints were included.  Information about the patient’s demographics, clinical characteristics, medical co-morbidities, medications, and neuropsychological testing reports were extracted. Performance on cognitive and psychological measures were considered impaired if they were reported as “impaired”, or “abnormal”. 

Of 192 patient records reviewed, 21 had past neuropsychological testing, of which 11 were available in the electronic medical record (mean age 47.6 (SD=15.7), mean disease duration 8.3 (SD=6.1) years, 8 (73%) female, 9 (82%) Caucasian). Of those with testing reports available, 7 (64%) patients had impairments in memory, 6 (55%) had impairments in attention, 6 (55%) in executive function, 6 (55%) in motor speed, 5 (54%) in processing speed, 4 (36%) in language and 2 (18%) in visuospatial skills. Seven (64%) had moderate-to-severe depressive or anxiety symptoms on neuropsychological evaluation, but only 5 (45%) were on antidepressants.

Evaluation of subjective cognitive complaints in SPS should include assessment of memory, attention, executive function, motor speed and processing speed. Moderate-to-severe depression and anxiety are common in SPS patients with cognitive impairment. Further understanding of neuropsychological symptoms in SPS is needed to identify interventions and improve quality of life in this population.

Authors/Disclosures
Scott D. Newsome, DO, FAAN (Johns Hopkins Hospital)
PRESENTER
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.
No disclosure on file
Yujie Wang, MD (UW Northwest) Dr. Wang has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics. The institution of Dr. Wang has received research support from Genentech. The institution of Dr. Wang has received research support from uniQure. The institution of Dr. Wang has received research support from NIH/NINDS.
No disclosure on file