Work

Understanding stiff-person syndrome: Epidemiological trends, diagnostic challenges, and treatment advances

Year: 2026

Type: article

Abstract: Stiff-person syndrome spectrum disorders (SPSD) comprise a group of rare neuroimmunological conditions characterized by fluctuating muscle stiffness, painful spasms, gait disturbance, and hypersensitivity triggers. Emerging phenotypes, including posterior fossa dysfunction, highlight the heterogeneity of the spectrum. Although historically described as a "one-in-a-million" disorder, recent epidemiologic data suggest that SPSD is more common than previously recognized. Currently, consensus diagnostic criteria do not exist; however, proposed criteria emphasize key clinical findings, high titers of recognized antibodies and electrophysiology findings that help support a diagnosis. The goal of this review is to provide an overview of SPSD epidemiology with emphasis on prevalence studies, emerging diagnostic criteria and overview of the multimodal treatment approach integrating pharmacological symptomatic therapies, immunotherapy, and non-pharmacologic interventions.

Source: Journal of Neuroimmunology

DOI: 10.1016/j.jneuroim.2026.578888

Authors: Daniela Riveros, Asli Buyukkurt, Scott D. Newsome

Institutions: Johns Hopkins University, Johns Hopkins Medicine

Language: English

Topic: Autoimmune Neurological Disorders and Treatments

Subfield: Neurology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: hybrid

Estimated APC paid: $3,360