Rituximab as Initial gMG Treatment, WVE-003 Proves Positive in HD, and Incidence of Guillian-Barré After COVID-19 Vaccination

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Welcome to this special edition of Neurology News Network. I’m Matt Hoffman, filling in for Marco Meglio. Let’s take a look at some of this week’s top news.

First up this week, the findings of a randomized, double-blind, placebo-controlled study of rituximab among individuals with generalized myasthenia gravis, or gMG, suggest that the treatment in a single 500-mg dose was associated with a greater chance of minimal disease manifestation and a reduction in rescue medication use. All told, of the 24 individuals randomly assigned to rituximab, 71% met the primary end point of minimal gMG manifestation—defined as a Quantitative Myasthenia Gravis score of 4 or less, being on 10 mg or less prednisolone, and not using rescue treatment—through week 16. Comparatively, of those in the placebo group, 29% met these criteria, a significant difference between the groups. Notably, in this trial, the treatment was used in a cohort of patients who had new-onset disease, with an onset of generalized symptoms within 12 months, and a QMG score of 6 or more.

Next up, new findings from the phase 1b/2a SELECT-HD trial of WVE-003, an investigational agent for Huntington disease from Wave Life Sciences, showed that the treatment reduced HD-specific mutant huntingtin protein in cerebrospinal fluid, as well as preserved wild-type huntingtin protein.1 All told, the stereopure antisense oligonucleotide was well-tolerated and safe for doses up to 90 mg. For those who received 30- and 60-mg single doses, pooled analysis showed a 22% median reduction in CSF mutant HTT from baseline to day 85, and an even greater difference in mean reduction of 35% when compared with placebo. Wave noted that additional single-dose biomarker and safety data are expected in the first half of 2023.

Finally, findings from the Vaccine Adverse Event Reporting System database presented at the 2022 American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM) annual meeting, in Nashville, Tennessee, showed that among pediatric patients, there is an increased prevalence of Guillain-Barré syndrome 6 weeks after receiving the Pzifer-BioNTech COVID-19 vaccination, suggesting a potential temporal association. The study compared the rate of pediatric Guillain-Barré following COVID-19 vaccination to the rate after influenza, HPV, and meningococcal vaccinations. All told, there were 31 cases of Guillain-Barré reported after COVID-19, compared with 3, 1, and 1 cases after the influenza, HPV, and meningococcal vaccinations, respectively. Between vaccinations, the reporting rate of cases after COVID-19 vaccination was significantly higher than the others, at 12.45 per 10 million followed by influenza, meningococcal, and HPV vaccinations. Notably, though, these rates for Guillain-Barré after COVID-19 vaccination were within the incidence rate of GBS typically reported in children.

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