2026 American Academy of Neurology Autoimmune Abstract Website

Yoji Hoshina¹, Ka-Ho Wong², Sadie McGreer¹, Justin Abbatemarco³, Bettina Balint⁴, Kyle Blackburn⁵, Stefan Blum⁶, Adrian Budhram⁷, Marinos Dalakas⁸, Josep Dalmau⁹, Livia Dutra¹⁰, Francesc Graus⁹, Jerome Honnorat¹¹, Takahiro Iizuka¹², Sarosh Irani¹³, Saiju Jacob¹⁴, Eric Lancaster¹⁵, Jenny Linnoila¹⁶, Alfonso Lopez¹³, Sara Mariotto¹⁷, Naoko Matsui¹⁸, Andrew McKeon¹³, Joao Moura¹⁹, Scott Newsome²⁰, Orna O'Toole²¹, Amanda Piquet²², Amy Quek²³, Albert Saiz²⁴, Mateus Simabukuro²⁵, Claudia Sommer²⁶, Maarten Titulaer²⁷, Alberto Vogrig²⁸, Yujie Wang²⁹, Tara Zier³⁰, Jacqueline Kraska³⁰, Giovanna Manzano³¹, Anastasia Zekeridou³², Stacey Clardy³³
¹University of Utah Health, ²U of U Neurology Clinic, ³Cleveland Clinic, ⁴University Hospital of Zurich, ⁵University of Texas Southwestern Medical Center, ⁶Princess Alexandra Hospital, ⁷London Health Sciences Centre, ⁸Thomas Jefferson University, ⁹IDIBAPS, ¹⁰Hospital Israelita Albert Einstein, ¹¹Hospices Civils de Lyon, ¹²Department of Neurology, Kitasato University School of Medicine, ¹³Mayo Clinic, ¹⁴University Hospitals Birmingham NHS Foundation Trust, ¹⁵The University of Pennsylvania, Dept. of Neurology, ¹⁶University Neurology Associates, UPMC, ¹⁷Neurology Unit, University of Verona, ¹⁸Tokushima University, ¹⁹Centro Hospitalar Universitário do Porto, ²⁰Johns Hopkins Hospital, ²¹Mercy University Hospital, ²²University of Colorado, ²³National University Hospital, ²⁴Hospital Clinico De Barcelona, ²⁵Hospital Das Clinicas, Sao Paulo U Scho of Med, ²⁶Neurologische Klinik der Universitat, ²⁷Erasmus Medical Center, ²⁸University of Udine, ²⁹UW Northwest, ³⁰The Stiff Person Syndrome Research Foundation, ³¹University of California Irvine, ³²Neuroimmunology Laboratory, Mayo Clinic, ³³University of Utah

Objective:

To establish expert consensus and identify practice variation in treatment and monitoring approaches for stiff person syndrome (SPS) and related disorders.

Background:

International consensus guidance on the treatment and monitoring of SPS and related disorders is lacking, and management approaches may vary across centers, countries, and resource settings.

Design/Methods:

Forty international experts in SPS were identified based on peer-reviewed authorship. A modified Delphi survey was designed to evaluate agreement on treatment and monitoring. Consensus was predefined as greater than 80% agreement.

Results:

Of 40 invited expert clinicians, 33 (83%) from 15 countries participated. Consensus was achieved for a 2-pronged treatment approach combining symptomatic therapy and immunotherapy in SPS. Consensus was also reached for benzodiazepines as first-line symptomatic treatment; adjunctive baclofen and botulinum toxin in selected patients; avoidance of opioids; use of intravenous immunoglobulin (IVIg) as first-line acute immunotherapy and first-line maintenance immunotherapy; plasma exchange for refractory or acutely worsening disease; individualized maintenance IVIg adjustment and tapering after sustained stability; rituximab for partial or nonresponders to IVIg; immunotherapy as the cornerstone of treatment for progressive encephalomyelitis with rigidity and myoclonus (PERM) and glutamic acid decarboxylase 65 (GAD65)-associated cerebellar ataxia and epilepsy; the preferred role of rituximab in glycine receptor alpha1 antibody-associated PERM; concomitant antiseizure therapy for seizure phenotypes; consideration of anti-CD19 chimeric antigen receptor T-cell therapy (CAR-T) for selected SPS patients; monitoring using objective functional measures; psychiatric screening; and diagnostic reassessment at follow-up. Consensus was not reached after Round 1 regarding initial IVIg dose and interval strategies, the use of hematopoietic stem-cell transplantation, pregnancy-specific treatment strategies, and several adjunctive symptomatic therapies.

Conclusions:

This international Delphi survey helps identify areas of expert agreement and variation in the treatment and monitoring of SPS and related disorders across diverse clinical settings. These findings, supplemented by the round 2 data, may help inform future consensus recommendations.