Beliefs and Attitudes of Neurology Clinicians on Stiff Person Syndrome: A National Survey on Diagnosis and Management
Divya Arya1, Yujie Wang1, Annette Wundes1, Gloria Von Geldern1, Gloria Hou1, Sarah Simmons1, Michael Persenaire1, Elisa McGee1, Wenxuan Xiong1, Shuvro Roy1
1University Of Washington
Objective:
To evaluate practicing and trainee neurologists' beliefs and attitudes regarding the diagnosis and management of Stiff Person Syndrome (SPS).
Background:
SPS is a rare and frequently misdiagnosed neurological disorder. This cross-sectional survey gathered neurology clinicians experiences with SPS diagnosis and management.
Design/Methods:

An online survey was distributed from 03/2024 to 08/2024 globally. USA based neurology residency and fellowship programs, as well as organizations such as the Consortium of Multiple Sclerosis Centers, SPS research foundation, Cleveland Clinic and University of Washington alumni also assisted in dissemination.

Results:
Of 126 respondents, 51% were trainees (residents and fellows) and 46% practicing neurologists. Practicing neurologists had a median of 10 years of post-training experience. Most respondents (75%) worked in academic centers, and 69% had seen fewer than 10 SPS patients. Only 17% were confident in diagnosing SPS. Twenty-six percent of respondents referred patients for subspecialty evaluation, most commonly to neuroimmunology. Fewer than 45% recognized clinical signs such as exaggerated startle reflex and hyperlordosis. For diagnostic testing, 80% ordered glutamic acid decarboxylase-65 (GAD65) antibodies, 49% tested other antibodies, 37% ordered CSF analysis, and 31% utilized electromyography. 50% of respondents identified the typical GAD65 antibody titers associated with neuroimmunity. In making a diagnosis, 39% were familiar with different proposed diagnostic criteria. 48% reported being unsure of identifying atypical presentation of SPS. Preferred drug choices most commonly reported were benzo/baclofen for symptom management (65%), IVIG for acute episodes (41%) and maintenance (39%). Non-pharmacological therapies such as massage, acupuncture etc. were recommended by 27% of respondents. Data from further multivariate analysis to associated practice patterns based on demographics will be presented at the AAN conference.

Conclusions:
Our findings highlight the need for improved education in diagnosing and managing SPS.
10.1212/WNL.0000000000211357
Disclaimer: Abstracts were not reviewed by Neurology® and do not reflect the views of Neurology® editors or staff.