Severe Stiff-Person Syndrome After COVID: The First Video-Documented COVID Exacerbation and Viral Implications.

Neurol Neuroimmunol Neuroinflamm

From the Department of Neurology, Thomas Jefferson University, Philadelphia, PA; Neuroimmunology Unit, National and Kapodistrian University of Athens Medical School, Athens, Greece.

Published: March 2024

Objectives: To describe a patient with mild GAD-positive stiff-leg syndrome (SLS) who developed severely disabling stiff-person syndrome (SPS) 1 week after mild COVID-19 and discuss the impact of viral implications.

Methods: Video-documented serial clinical observations at baseline, after acute COVID-19, and after IVIG treatments.

Results: A 39-year-old man with left-SLS was stable during a 2-year follow-up with low-dose antispasmodics, working fully and functioning normally, even able to run. One week after mild COVID-19, he started to experience generalized SPS symptomatology that steadily worsened the following 2-3 weeks, becoming unable to walk, requiring a walker, with significant thoracolumbar and bilateral leg stiffness and spasms. GAD ab were very high. After 3 monthly IVIg infusions he showed improvements, but his gait remains significantly stiff. All clinical changes, from baseline to post-Covid, and then post- IVIg have been video-documented.

Discussion: This is the first, clearly documented, severe GAD-positive SPS after COVID-19. Although viral or postviral causation can be incidental, the temporal connection with acute COVID-19, the severe disease worsening after symptom-onset, and the subsequent steady improvement after IVIg, suggest viral-triggered autoimmunity. Because COVID-19 reportedly can trigger or worsen GAD-associated diabetes type 1 through proinflammatory mediators, and SPS has been reportedly triggered by West Nile Virus, possibly through molecular mimicry, this case of acutely converting GAD-SLS to GAD-SPS suggest the need to explore viral etiologies in patients with GAD-SPS experiencing acute, long-lasting episodic exacerbations of stiffness and spasms.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10751017PMC
http://dx.doi.org/10.1212/NXI.0000000000200192DOI Listing

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