Severe Stiff-Person Syndrome After COVID

Abstract

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.

Reply: Infections and Stiff‐Person Spectrum Disorders

“…Recently, Dalakas reported a case of a 39-year-old man who initially presented with stable and mild left stiff leg syndrome (SLS) that progressed to severe generalized SPS 1 week after the onset of COVID-19. 5 In all three cases, the short interval between infection and neurological manifestation may be attributed to a latent autoimmune response triggered by the virus, thereby initiating or exacerbating the disease. This phenomenon is documented in several post-COVID-19 autoimmune diseases, such as Guillain-Barré syndrome (GBS), which occurs immediately after the infection.…”

“…This phenomenon is documented in several post-COVID-19 autoimmune diseases, such as Guillain-Barré syndrome (GBS), which occurs immediately after the infection. 5 Conversely, given the short interval and very low frequency of the COVID-19 and SPS association, it appears less likely that the virus is directly implicated in the pathogenetically specific autoimmune response of SPS.…”