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May 15, 2020
Guillain-Barre Syndrome, a Potential Complication of COVID-19
Jaime Toro, MD reviewing Toscano G et al. N Engl J Med 2020 Apr 17
Guillain-Barre is a neurologic complications observed in association with SARS-CoV-2.
The major manifestations of COVID-19 are pulmonary. Nevertheless, neurologic disease may result, rarely through direct infection, less rarely through parainfectious complications, or more commonly via critical illness. In this series, five cases of Guillain-Barre syndrome (GBS) in patients with COVID-19 were seen in three hospitals in northern Italy from February 28 through March 21, 2020.
The first symptoms of GBS were lower limb weakness in four patients and facial diplegia with subsequent ataxia and paresthesia in one patient. Four patients had generalized flaccid tetraparesis or tetraplegia that developed over 3 to 4 days; three of these patients received mechanical ventilation. GBS symptoms began 5 to 10 days after the onset of COVID-19 symptoms. No patient had dysautonomic features. Protein levels in the cerebrospinal fluid (CSF), measured in two patients, were normal. In all five patients, white cell count was less than 5 per mm3 in all patients and real-time polymerase chain reaction assay of the CSF was negative for SARS-CoV-2. Electrophysiologic study results were consistent with axonal variant of GBS in three patients and with demyelination in two. All patients were treated with intravenous immune globulin and one also received plasma exchange. After 4 weeks of treatment, two patients remained in intensive care, two were receiving physical therapy, and one was discharged walking independently.
Muscle Nerve
2020 Oct;62(4):485-491. doi: 10.1002/mus.27024. Epub 2020 Aug 11.
COVID-19-associated Guillain-Barre syndrome:
The early pandemic experience
James B Caress 1, Ryan J Castoro 1, Zachary Simmons 2, Stephen N Scelsa 3, Richard A Lewis 4, Aditi Ahlawat 5, Pushpa Narayanaswami 5 Affiliations expand PMID: 32678460 PMCID: PMC7405390 DOI: 10.1002/mus.27024Abstract
Guillain-Barre syndrome (GBS) is an inflammatory polyradiculoneuropathy associated with numerous viral infections. Recently, there have been many case reports describing the association between coronavirus disease-2019 (COVID-19) and GBS, but much remains unknown about the strength of the association and the features of GBS in this setting. We reviewed 37 published cases of GBS associated with COVID-19 to summarize this information for clinicians and to determine whether a specific clinical or electrodiagnostic (EDx) pattern is emerging. The mean age (59 years), gender (65% male), and COVID-19 features appeared to reflect those of hospitalized COVID-19 patients early in the pandemic. The mean time from COVID-19 symptoms to GBS symptoms was 11 days. The clinical presentation and severity of these GBS cases was similar to those with non-COVID-19 GBS. The EDx pattern was considered demyelinating in approximately half of the cases. Cerebrospinal fluid, when assessed, demonstrated albuminocytologic dissociation in 76% of patients and was negative for severe acute respiratory distress syndrome-coronavirus-2 (SARS-CoV-2) in all cases. Serum antiganglioside antibodies were absent in 15 of 17 patients tested. Most patients were treated with a single course of intravenous immunoglobulin, and improvement was noted within 8 weeks in most cases. GBS-associated COVID-19 appears to be an uncommon condition with similar clinical and EDx patterns to GBS before the pandemic. Future studies should compare patients with COVID-19-associated GBS to those with contemporaneous non-COVID-19 GBS and determine whether the incidence of GBS is elevated in those with COVID-19.
SARS-CoV-2 Vaccination Safety in Guillain-Barré Syndrome, Chronic Inflammatory Demyelinating Polyneuropathy, and Multifocal Motor Neuropathy
Adája E. Baars, Krista Kuitwaard, Laura C. de Koning, Linda W.G. Luijten, W. Maaike Kok, Filip Eftimov, Luuk Wieske, H. Stephan Goedee, W. Ludo van der Pol, Patricia H. Blomkwist-Markens, Anja M.C. Horemans, Bart C. Jacobs, Pieter A. van Doorn First published September 20, 2022, DOI: https://doi.org/10.1212/WNL.0000000000201376kground 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. Glossary CIDP=chronic inflammatory demyelinating polyneuropathy; GBS=Guillain-Barré syndrome; IVIg=intravenous immunoglobulins; MMN=multifocal motor neuropathy The introduction of SARS-CoV-2 vaccinations is an important milestone in the COVID-19 pandemic. However, patients with a history of an immune-mediated neuropathy are often concerned about the safety of vaccinations because of a possible recurrence of Guillain-Barré syndrome (GBS) or exacerbations of chronic inflammatory demyelinating polyneuropathy (CIDP) or multifocal motor neuropathy (MMN).1,2 GBS usually is a monophasic disease with severe muscle weakness, which is followed by recovery to a variable extent and duration.3 CIDP and MMN are chronic polyneuropathies requiring long-term treatment, usually with intravenous immunoglobulins (IVIg) or corticosteroids. In SARS-CoV-2 vaccination phase III trials, individuals with a prior diagnosis of GBS or receiving IVIg or systemic corticosteroids within months before study vaccine administration were excluded from participation.4,-,7 Therefore, data from these trials regarding safety of SARS-CoV-2 vaccinations cannot instantaneously be extrapolated to most of the patients with these immune-mediated neuropathies. Concerns regarding safety of vaccination and immune-mediated neuropathies stem from a described eightfold increased incidence rate of GBS after vaccination during the H1N1 influenza vaccination campaign in 1976.8,9 Similar correlations have not been observed since, although temporal associations between vaccination and GBS have been reported.10,-,13 GBS has an estimated life-time recurrence risk between 3.5% and 6.6%.12,14,-,17 No GBS recurrences were reported within 6 weeks after seasonal flu vaccination or after various other vaccines, such as pneumovax and Hepatitis A and B.17,18 The pathophysiologic mechanisms between vaccinations and the occurrence of GBS after vaccinations however remain unclear.19 Despite these more recent studies,20 GBS is still listed as an adverse event of special interest in pharmacovigilance studies to ensure early detection of a potential association with any new vaccine. For CIDP and MMN, no increased incidence has been reported after any vaccination, such as seasonal flu vaccination. In a retrospective study, 3 of 65 patients with CIDP reported that they had experienced symptoms similar to a typical relapse of CIDP after receiving a vaccination.12 In the general population, an increased incidence of GBS in the 28 days after vaccination with ChadOx1 nCoV-19 (AstraZeneca) has been observed.21 After increased signals of GBS incidence in passive reporting systems, safety warnings were issued for both ChadOx1 nCoV-19 (AstraZeneca) and Ad26.COV2.S (Janssen/Johnson & Johnson).22,23 However, an association between SARS-CoV-2 infection and an increased incidence of GBS has also been described, and this association was much stronger than for SARS-CoV-2 vaccinations.21,24 This highlights the value of preventing SARS-CoV-2 infections, especially in patients with immune-mediated neuropathies, if SARS-CoV-2 vaccines can be safely administered in patients who have had GBS. The scale of the SARS-CoV-2 vaccination campaigns created a unique opportunity to investigate the possible relationship between SARS-CoV-2 vaccination and the course of disease in immune-mediated neuropathies. The objective of this study was to explore the risk of recurrence of GBS or worsening of disease-related symptoms in CIDP and MMN after SARS-CoV-2 vaccination.