SARS-CoV-2 Vaccination Safety in Guillain-Barré Syndrome, Chronic Inflammatory Demyelinating Polyneuropathy, and Multifocal Motor Neuropathy.

BACKGROUND AND OBJECTIVES: There are concerns on the safety of SARS-CoV-2 vaccination in patients with a history of Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), and multifocal motor neuropathy (MMN). The aim of this study was to determine the risk of recurrence of GBS and exacerbations of CIDP or MMN after SARS-CoV-2 vaccination. METHODS: We conducted a prospective, multicenter cohort study from January 2021 to August 2021. Patients known in 1 of 3 Dutch University Medical Centers with research focus on immune-mediated neuropathy and members of the Dutch Patient Association for Neuromuscular Diseases were invited to participate if they were 18 years or older and diagnosed with GBS, CIDP, or MMN. Participants completed a series of questionnaires at 4 different time points: study baseline (1), within 48 hours before any SARS-CoV-2 vaccination (2 and 3, if applicable), and 6 weeks after their last vaccination (4). Participants unwilling to get vaccinated completed the last questionnaire (4) 4 months after study baseline. We assessed recurrences of GBS, any worsening of CIDP or MMN-related symptoms, treatment alterations, and hospitalization. RESULTS: Of 1,152 individuals to whom we sent the questionnaires, 674 (59%) signed informed consent. We excluded 153 individuals, most often because they had already received a SARS-CoV-2 vaccination or had had the infection (84%) before study baseline. Of 521 participants included in analyses, 403 (81%) completed the last questionnaire (time point 4). None of 162 participants with a history of GBS had a recurrence after vaccination. Of 188 participants with CIDP, 10 participants (5%) reported a worsening of symptoms within 6 weeks after vaccination. In 5 (3%) of these patients, maintenance treatment was modified. Two of 53 participants with MMN (4%) reported a worsening of symptoms, and treatment modification was reported by 1 participant. DISCUSSION: We found no increased risk of GBS recurrence and a low to negligible risk of worsening of CIDP or MMN-related symptoms after SARS-CoV-2 vaccination. Based on our data, SARS-CoV-2 vaccination in patients with these immune-mediated neuropathies seems to be safe.

Incidence, Prevalence and Geographical Clustering of Motor Neuron Disease in the Netherlands.

OBJECTIVE: To assess time trends in MND incidence, prevalence and mortality and investigate geographical clustering of MND cases in the Netherlands from 1998 to 2017, we analyzed data from the Netherlands Personal Records database, the Netherlands MND Center and the Netherlands Patient Association of Neuromuscular Diseases. METHODS: In this prospective cohort study, Poisson regression was used to assess time trends in MND risk. We calculated age- and sex-standardized, observed and expected cases for 1,694 areas. Bayesian smoothed risk mapping was used to investigate geographical MND risk. RESULTS: We identified 7,992 MND cases, reflecting an incidence of 2.64 (95% CI 2.62-2.67) per 100,000 person-years and a prevalence of 9.5 (95% CI 9.1-10.0) per 100,000 persons. Highest age-standardized prevalence and mortality rates occurred at a later age in men than in women (p<0.001). Unadjusted mortality rates increased by 53.2% from 2.57 in 1998 to 3.86 per 100,000 person-years in 2017. After adjustment for age and sex, an increase in MND mortality rate of 14.1% (95% CI 5.7%-23.2%, p<0.001) remained. MND relative risk ranged from 0.78 to 1.43 between geographical areas; multiple urban and rural high-risk areas were identified. CONCLUSIONS: We found a significant national increase in MND mortality from 1998 through 2017, only partly explained by an ageing Dutch population, and also a geographic variability in MND risk, suggesting a role for environmental or demographic risk factors.

Research priorities of patients with neuromuscular disease.

PURPOSE: This paper describes a study in which patients with neuromuscular disease (NMD) were engaged to list top-priorities for scientific research in order to complement the researchers' agenda. METHOD: A dialogic model for research agenda setting was used. Interviews, focus groups and expert meetings with patients were held to identify research topics. Research topics were prioritized via a questionnaire. Agendas were integrated in a dialogue meeting with professionals and patients. RESULTS: The research agenda of NMD patients is divided in four research domains, with a total of 24 research topics. These domains include (1) health; (2) quality of life; (3)quality of care and support, and (4) basic issues. Among the research domains highest priority was given to research on health, followed by research on quality of life. CONCLUSIONS: Both patients and professionals agreed a proper balance needs to be found between fundamental research and research on symptomatic treatment and quality of life. They concluded that more attention is required for research on the effective treatment of symptoms, quality of life and implementation of knowledge about NMD in regular care.