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Publications (10 of 79) Show all publications
O'Connor, L., Malmestrom, C., Rodrigues, R. D., Brauner, S., Wikström, A.-K. & Rostedt Punga, A. (2024). Pregnancy outcomes for women with myasthenia gravis and their newborns: A nationwide register-based cohort study. European Journal of Neurology, 31(1)
Open this publication in new window or tab >>Pregnancy outcomes for women with myasthenia gravis and their newborns: A nationwide register-based cohort study
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2024 (English)In: European Journal of Neurology, ISSN 1351-5101, E-ISSN 1468-1331, Vol. 31, no 1Article in journal (Refereed) Published
Abstract [en]

Background and Purpose: Few large-scale studies examine whether maternal myasthenia gravis (MG) is a risk factor for complications during pregnancy and childbirth. This study evaluated whether maternal MG is associated with an increased risk of adverse pregnancy, delivery, and neonatal outcomes.

Methods: We conducted a nationwide Swedish register-based cohort study of women who gave birth to singleton infants (>= 22 gestational weeks) during 1987-2019. Exposed women were diagnosed with MG before or during the index pregnancy (N = 443). Unexposed women comprised 4249 women without a diagnosis of MG, matched for age, parity, hospital, and year of childbirth. The risks of adverse pregnancy, delivery, and neonatal outcomes for women with MG were estimated using regression modeling and presented as adjusted odds ratios (aOR).

Results :There was no increased risk of pregnancy complications in women with MG. Women with MG had a spontaneous onset of labor less often than women without MG (69.8% vs. 79.5%; aOR 0.59; p < 0.001) as well as higher labor induction rates and elective cesarean section deliveries (16.0% vs. 12.3%, aOR 1.42; p = 0.02 and 12.0% vs. 8.1%, aOR 1.59; p = 0.009). Infants of women with MG were born on average 2 days earlier (p = 0.002); however, these infants did not have a higher risk of having low APGAR, being small for gestational age, or having a congenital malformation.

Conclusion :This first nationwide study of pregnancy in women with MG in Sweden demonstrates reassuring results overall, suggesting generally safe pregnancy outcomes for women with MG and their infants.

Place, publisher, year, edition, pages
John Wiley & Sons, 2024
Keywords
myasthenia gravis, newborn, postpartum, pregnancy
National Category
Obstetrics, Gynecology and Reproductive Medicine
Identifiers
urn:nbn:se:uu:diva-528422 (URN)10.1111/ene.16100 (DOI)001085109600001 ()37843262 (PubMedID)
Funder
Swedish Research Council, VR-523-2014-2048
Available from: 2024-05-24 Created: 2024-05-24 Last updated: 2024-05-24Bibliographically approved
O'Connor, L., Wikström, A.-K. & Rostedt Punga, A. (2024). Response to "More evidence is needed for the association between serum myasthenia gravis and adverse pregnancy, delivery and neonatal outcomes" [Letter to the editor]. European Journal of Neurology, 31(3), Article ID e16162.
Open this publication in new window or tab >>Response to "More evidence is needed for the association between serum myasthenia gravis and adverse pregnancy, delivery and neonatal outcomes"
2024 (English)In: European Journal of Neurology, ISSN 1351-5101, E-ISSN 1468-1331, Vol. 31, no 3, article id e16162Article in journal, Letter (Other academic) Published
Place, publisher, year, edition, pages
John Wiley & Sons, 2024
National Category
Neurology
Identifiers
urn:nbn:se:uu:diva-533661 (URN)10.1111/ene.16162 (DOI)001109266500001 ()37990801 (PubMedID)
Available from: 2024-07-03 Created: 2024-07-03 Last updated: 2024-07-03Bibliographically approved
Kosek, S., Persson, B., Rodrigues, R., Malmestrom, C., Rostedt Punga, A. & Burman, J. (2023). Antibody-Positive Autoimmune Encephalitis and Paraneoplastic Neurological Syndrome: Epidemiology and Outcome of Neuronal Antibody Testing in Sweden. Acta Neurologica Scandinavica, 2023, Article ID 6993615.
Open this publication in new window or tab >>Antibody-Positive Autoimmune Encephalitis and Paraneoplastic Neurological Syndrome: Epidemiology and Outcome of Neuronal Antibody Testing in Sweden
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2023 (English)In: Acta Neurologica Scandinavica, ISSN 0001-6314, E-ISSN 1600-0404, Vol. 2023, article id 6993615Article in journal (Refereed) Published
Abstract [en]

Objective. To estimate the 5-year incidence rate of autoimmune encephalitis (AE) and paraneoplastic neurological syndrome (PNS) in Sweden. Methods. All patients who were tested for a neuronal antibody in Sweden between 2015 and 2019 were included. Patients in Healthcare region Mid Sweden (population 2.1 million) were invited to participate in a case ascertainment substudy. AE and PNS cases were defined using established diagnostic criteria. Crude and age-adjusted incidence rates of AE and PNS in Healthcare region Mid Sweden were estimated. Results. The number of tests for neuronal antibodies in Sweden increased between 2015 and 2019 from 1867 to 2505 (serum) and 863 to 1376 (CSF) per annum. The frequencies of positive results were stable over the entire study period, and the mean value was 6.1% for serum (CI95% 5.5-6.7) and 4.8% for CSF (CI95% 4.0-5.6). In total, 125 patients tested positive for neuronal antibodies in Healthcare region Mid Sweden between 2015 and 2019. Of these, 94 were included, and after case ascertainment, thirty-one cases of definite AE or PNS could be identified. The 5-year incidence rate of AE and PNS was 3.0 per million person-years (95% CI 1.9-4.1). The yearly incidence rates increased in the study period, from 1.5 per million person-years in 2015 (95% CI 0.0-3.2) to 4.3 per million person-years in 2019 (95% CI 1.5-7.1). Conclusions. In this first epidemiological study of AE and PNS in Sweden, the number of cases doubled from 2015 to 2019. This likely reflects increased availability of testing and awareness of these conditions.

Place, publisher, year, edition, pages
WILEY, 2023
National Category
Medical and Health Sciences
Identifiers
urn:nbn:se:uu:diva-502110 (URN)10.1155/2023/6993615 (DOI)000974734400001 ()
Funder
Swedish Research Council, VR-523-2014-2048Marcus Wallenbergs Foundation for International Scientific CollaborationSwedish Society of Medicine
Available from: 2023-06-28 Created: 2023-06-28 Last updated: 2023-06-28Bibliographically approved
Rostedt Punga, A., Westerberg, E. & Åsenlöf, P. (2023). Implementation of tailored exercise programs for MG patients in a gym setting: a pragmatic feasibility case study. Neuromuscular Disorders, 33(4), 334-338
Open this publication in new window or tab >>Implementation of tailored exercise programs for MG patients in a gym setting: a pragmatic feasibility case study
2023 (English)In: Neuromuscular Disorders, ISSN 0960-8966, E-ISSN 1873-2364, Vol. 33, no 4, p. 334-338Article in journal (Refereed) Published
Abstract [en]

Although supervised aerobic and resistance training in a hospital setting was proven safe and beneficial for well-controlled myasthenia gravis (MG) patients, implementation of similar programs in the community has not been studied. We conducted a pragmatic open-label study at a large gym in Uppsala, Sweden. Seven patients with generalized MG were recruited to participate in an individualized, tailored exercise program, based on individual baseline status and personal goals, with a personal trainer. All patients completed the entire training period. The individually tailored exercise program was implemented safely and effectively, with all patients improving in aerobic capacity, muscle strength, and balance. Our pragmatic open-label case study suggests that well-controlled patients with generalized MG can extend their physical exercise to personal training in the gym. This is an essential step towards reducing the barriers to implementing exercise procols and increasing the availability of these interventions to MG patients.

Place, publisher, year, edition, pages
Elsevier, 2023
Keywords
Physical exercise, Myasthenia gravis, MG, Tailored exercise, Personal training, Pragmatic study
National Category
Physiotherapy Neurology
Identifiers
urn:nbn:se:uu:diva-500580 (URN)10.1016/j.nmd.2023.02.009 (DOI)000956594900001 ()36931100 (PubMedID)
Funder
Swedish Research Council, VR-523-2014-2048Erik, Karin och Gösta Selanders FoundationSwedish Association of Persons with Neurological Disabilities
Available from: 2023-04-24 Created: 2023-04-24 Last updated: 2023-04-24Bibliographically approved
Rostedt Punga, A., Alimohammadi, M. & Liik, M. (2023). Keeping up appearances: Don't frown upon the effects of botulinum toxin injections in facial muscles. Clinical Neurophysiology Practice, 8, 169-173
Open this publication in new window or tab >>Keeping up appearances: Don't frown upon the effects of botulinum toxin injections in facial muscles
2023 (English)In: Clinical Neurophysiology Practice, E-ISSN 2467-981X, Vol. 8, p. 169-173Article, review/survey (Refereed) Published
Abstract [en]

Aesthetic use of low doses of Botulinum toxin (BoNT) injections into the facial muscles has become a leading non-surgical aesthetic treatment worldwide to reduce facial wrinkles, including glabellar lines, forehead lines, and periorbital wrinkles. Within these aesthetic applications, BoNT injections intend to reduce and prevent wrinkles, and the recommended usage of 2 years is often exceeded, which may result in atrophy of the injected muscles. The long-term effects of BoNT injections in the facial muscles and the evidence of diffusion of BoNT to surrounding muscles are obvious pitfalls and challenges for clinical neurophysiologists in differential diagnosing neuromuscular transmission failures. Also, this is further complicated by the risk of developing side effects upon permanent chemical denervation of facial muscles, with less possibility for reinnervation.

This review summarizes the known long-term effects of BoNT over time in different facial muscles and the use of objective electrophysiological measures to evaluate these. A better understanding of the longterm effects of BoNT is essential to avoid misdiagnosing other neuromuscular disorders.

Place, publisher, year, edition, pages
Elsevier, 2023
Keywords
Botulinum toxin, Neuromuscular, Long -term effects, Cosmetic
National Category
Physiology Dermatology and Venereal Diseases Neurosciences
Identifiers
urn:nbn:se:uu:diva-514145 (URN)10.1016/j.cnp.2023.05.005 (DOI)001075805600001 ()37681120 (PubMedID)
Available from: 2023-10-17 Created: 2023-10-17 Last updated: 2023-10-17Bibliographically approved
Meisel, A., Baggi, F., Behin, A., Evoli, A., Kostera-Pruszczyk, A., Mantegazza, R., . . . Leite, M.-I. (2023). Reply to the Letter to the Editor in response to "Role of autoantibody levels as biomarkers in the management of patients with myasthenia gravis: A systematic review and expert appraisal " [Letter to the editor]. European Journal of Neurology, 30(4), 1162-1164
Open this publication in new window or tab >>Reply to the Letter to the Editor in response to "Role of autoantibody levels as biomarkers in the management of patients with myasthenia gravis: A systematic review and expert appraisal "
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2023 (English)In: European Journal of Neurology, ISSN 1351-5101, E-ISSN 1468-1331, Vol. 30, no 4, p. 1162-1164Article in journal, Letter (Other academic) Published
Place, publisher, year, edition, pages
John Wiley & Sons, 2023
Keywords
activity, autoantibody level, biomarker, myasthenia gravis
National Category
Neurology
Identifiers
urn:nbn:se:uu:diva-502444 (URN)10.1111/ene.15690 (DOI)000933229900001 ()36692238 (PubMedID)
Available from: 2023-05-25 Created: 2023-05-25 Last updated: 2023-05-25Bibliographically approved
Meisel, A., Baggi, F., Behin, A., Evoli, A., Kostera-Pruszczyk, A., Mantegazza, R., . . . Leite, M.-I. (2023). Role of autoantibody levels as biomarkers in the management of patients with myasthenia gravis: A systematic review and expert appraisal. European Journal of Neurology, 30(1), 266-282
Open this publication in new window or tab >>Role of autoantibody levels as biomarkers in the management of patients with myasthenia gravis: A systematic review and expert appraisal
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2023 (English)In: European Journal of Neurology, ISSN 1351-5101, E-ISSN 1468-1331, Vol. 30, no 1, p. 266-282Article, review/survey (Refereed) Published
Abstract [en]

Background and purpose Although myasthenia gravis (MG) is recognized as an immunoglobulin G autoantibody-mediated disease, the relationship between autoantibody levels and disease activity in MG is unclear. We sought to evaluate this landscape through systematically assessing the evidence, testing the impact of predefined variables on any relationship, and augmenting with expert opinion. Methods In October 2020, a forum of leading clinicians and researchers in neurology from across Europe (Expert Forum for Rare Autoantibodies in Neurology in Myasthenia Gravis) participated in a series of virtual meetings that took place alongside the conduct of a systematic literature review (SLR). Results Forty-two studies were identified meeting inclusion criteria. Of these, 10 reported some correlation between a patient's autoantibody level and disease severity. Generally, decreased autoantibody levels (acetylcholine receptor, muscle-specific kinase, and titin) were positively and significantly correlated with improvements in disease severity (Quantitative Myasthenia Gravis score, Myasthenia Gravis Composite score, Myasthenia Gravis Activities of Daily Living score, Myasthenia Gravis Foundation of America classification). Given the limited evidence, testing the impact of predefined variables was not feasible. Conclusions This first SLR to assess whether a correlation exists between autoantibody levels and disease activity in patients with MG has indicated a potential positive correlation, which could have clinical implications in guiding treatment decisions. However, in light of the limited and variable evidence, we cannot currently recommend routine clinical use of autoantibody level testing in this context. For now, patient's characteristics, clinical disease course, and laboratory data (e.g., autoantibody status, thymus histology) should inform management, alongside patient-reported outcomes. We highlight the need for future studies to reach more definitive conclusions on this relationship.

Place, publisher, year, edition, pages
John Wiley & Sons, 2023
Keywords
autoantibodies, biomarkers, myasthenia gravis
National Category
Neurology
Identifiers
urn:nbn:se:uu:diva-502446 (URN)10.1111/ene.15565 (DOI)000864744200001 ()36094738 (PubMedID)
Available from: 2023-05-25 Created: 2023-05-25 Last updated: 2023-05-25Bibliographically approved
Piehl, F., Eriksson-Dufva, A., Budzianowska, A., Feresiadou, A., Hansson, W., Hietala, M. A., . . . Frisell, T. (2022). Efficacy and Safety of Rituximab for New-Onset Generalized Myasthenia Gravis The RINOMAX Randomized Clinical Trial. JAMA Neurology, 79(11), 1105-1112
Open this publication in new window or tab >>Efficacy and Safety of Rituximab for New-Onset Generalized Myasthenia Gravis The RINOMAX Randomized Clinical Trial
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2022 (English)In: JAMA Neurology, ISSN 2168-6149, E-ISSN 2168-6157, Vol. 79, no 11, p. 1105-1112Article in journal (Refereed) Published
Abstract [en]

IMPORTANCE Rituximab is a third-line option for refractory generalized myasthenia gravis (MG) based on empirical evidence, but its effect in new-onset disease is unknown. OBJECTIVE To investigate the efficacy and safety of rituximab compared with placebo as an add-on to standard of care for MG. DESIGN, SETTING, AND PARTICIPANTS This randomized, double-blind, placebo-controlled study took place throughout 48 weeks at 7 regional clinics in Sweden. Key inclusion criteria were age older than 18 years, onset of generalized symptoms within 12 months or less, and a Quantitative Myasthenia Gravis (QMG) score of 6 or more. Patients were screened from October 20, 2016, to March 2, 2020. Key exclusion criteria included pure ocular MG, suspected thymoma, previous thymectomy, and prior noncorticosteroid immunosuppressants or high doses of corticosteroids. INTERVENTIONS Participants were randomized 1:1 without stratification to a single intravenous infusion of 500 mg of rituximab or matching placebo. MAIN OUTCOMES AND MEASURES Minimal disease manifestations at 16 weeks defined as a QMG score of 4 or less with prednisolone, 10 mg or less daily, and no rescue treatment. RESULTS Of 87 potentially eligible patients, 25 were randomized to rituximab (mean [SD] age, 67.4 [13.4] years; 7 [28%] female) and 22 to placebo (mean [SD] age, 58 [18.6] years; 7 [32%] female). Compared with placebo, a greater proportion with rituximab met the primary end point; 71% (17 of 24) in the rituximab group vs 29% (6 of 21) in the placebo group (Fisher exact test P = .007; probability ratio, 2.48 [95% CI, 1.20-5.11]). Secondary end points, comparing changes in Myasthenia Gravis Activities of Daily Living and Myasthenia Gravis Quality of Life at 16 weeks with QMG at 24 weeks did not differ between groups with censoring for rescue treatment (per-protocol analysis) but were in favor of active treatment when rescue treatment was taken into account by worst rank imputation (post hoc analysis). Rescue treatments were also more frequent in the placebo arm (rituximab: 1 [4%]; placebo, 8 [36%]). One patient in the placebo arm had a myocardial infarction with cardiac arrest and 1 patient in the active arm experienced a fatal cardiac event. CONCLUSIONS AND RELEVANCE A single dose of 500 mg of rituximab was associated with greater probability of minimal MG manifestations and reduced need of rescue medications compared with placebo. Further studies are needed to address long-term benefit-risk balance with this treatment.

Place, publisher, year, edition, pages
American Medical Association (AMA)American Medical Association (AMA), 2022
National Category
Neurology
Identifiers
urn:nbn:se:uu:diva-496842 (URN)10.1001/jamaneurol.2022.2887 (DOI)000857176500002 ()36121672 (PubMedID)
Available from: 2023-02-23 Created: 2023-02-23 Last updated: 2024-01-15Bibliographically approved
Vergoossen, D. L. E., Ruiter, A. M., Keene, K. R., Niks, E. H., Tannemaat, M. R., Strijbos, E., . . . Borgesf, L. S. (2022). Enrichment of serum IgG4 in MuSK myasthenia gravis patients. Journal of Neuroimmunology, 373, Article ID 577978.
Open this publication in new window or tab >>Enrichment of serum IgG4 in MuSK myasthenia gravis patients
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2022 (English)In: Journal of Neuroimmunology, ISSN 0165-5728, E-ISSN 1872-8421, Vol. 373, article id 577978Article in journal (Refereed) Published
Abstract [en]

Muscle-specific kinase (MuSK) myasthenia gravis (MG) is a neuromuscular autoimmune disease belonging to a growing group of IgG4 autoimmune diseases (IgG4-AIDs), in which the majority of pathogenic autoantibodies are of the IgG4 subclass. The more prevalent form of MG with acetylcholine receptor (AChR) antibodies is caused by IgG1-3 autoantibodies. A dominant role for IgG4 in autoimmune disease is intriguing due to its antiinflammatory characteristics. It is unclear why MuSK autoantibodies are predominantly IgG4. We hypothesized that MuSK MG patients have a general predisposition to generate IgG4 responses, therefore resulting in high levels of circulating IgG4. To investigate this, we quantified serum Ig isotypes and IgG subclasses using nephelometric and turbidimetric assays in MuSK MG and AChR MG patients not under influence of immunosuppressive treatment. Absolute serum IgG1 was increased in both MuSK and AChR MG patients compared to healthy donors. In addition, only MuSK MG patients on average had significantly increased and enriched serum IgG4. Although more MuSK MG patients had elevated serum IgG4, for most the IgG4 serum levels fell within the normal range. Correlation analyses suggest MuSK-specific antibodies do not solely explain the variation in IgG4 levels. In conclusion, although serum IgG4 levels are slightly increased, the levels do not support ubiquitous IgG4 responses in MuSK MG patients as the underlying cause of dominant IgG4 MuSK antibodies.

Place, publisher, year, edition, pages
Elsevier, 2022
Keywords
Immunoglobulins, IgG4, Autoimmune disease, Serum, MuSK, Myasthenia gravis
National Category
Clinical Laboratory Medicine Immunology in the medical area
Identifiers
urn:nbn:se:uu:diva-490010 (URN)10.1016/j.jneuroim.2022.577978 (DOI)000882616500007 ()36240543 (PubMedID)
Available from: 2022-12-07 Created: 2022-12-07 Last updated: 2023-02-07Bibliographically approved
Rostedt Punga, A., Maddison, P., Heckmann, J. M., Guptill, J. & Evoli, A. (2022). Epidemiology, diagnostics, and biomarkers of autoimmune neuromuscular junction disorders. Lancet Neurology, 21(2), 176-188
Open this publication in new window or tab >>Epidemiology, diagnostics, and biomarkers of autoimmune neuromuscular junction disorders
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2022 (English)In: Lancet Neurology, ISSN 1474-4422, E-ISSN 1474-4465, Vol. 21, no 2, p. 176-188Article, review/survey (Refereed) Published
Abstract [en]

Autoimmune neuromuscular junction disorders are rare. However, myasthenia gravis is being increasingly recognised in people older than 50 years. In the past 5-10 years, epidemiological studies worldwide suggest an incidence of acetylcholine receptor antibody-positive myasthenia gravis of up to 29 cases per 1 million people per year. Muscle-specific tyrosine kinase antibody-positive myasthenia gravis and Lambert-Eaton myasthenic syndrome are about 20 times less common. Several diagnostic methods are available for autoimmune neuromuscular junction disorders, including serological antibody, electrophysiological, imaging, and pharmacological tests. The course of disease can be followed up with internationally accepted clinical scores or patient-reported outcome measures. For prognostic purposes, determining whether the disease is paraneoplastic is of great importance, as myasthenia gravis can be associated with thymoma and Lambert-Eaton myasthenic syndrome with small-cell lung cancer. However, despite well defined diagnostic parameters to classify patients into subgroups, objective biomarkers for use in the clinic or in clinical trials to predict the course of myasthenia gravis and Lambert-Eaton myasthenic syndrome are needed.

Place, publisher, year, edition, pages
ElsevierElsevier BV, 2022
National Category
Neurology
Identifiers
urn:nbn:se:uu:diva-472222 (URN)10.1016/S1474-4422(21)00297-0 (DOI)000769111800018 ()35065040 (PubMedID)
Available from: 2022-04-12 Created: 2022-04-12 Last updated: 2024-01-15Bibliographically approved
Projects
Elucidation of miRNAs in model diseases of autoimmune etiology and neuromuscular transmission [2014-02048_VR]; Uppsala UniversityElucidation of miRNAs in model diseases of autoimmune etiology and neuromuscular transmission [2014-07603_VR]; Uppsala UniversityNovel microtechnology-based cell model system to study human neurological diseases [2016-02184_VR]; Uppsala UniversityMicrotechnology system for human autoimmune and viral neurological disorders [2020-02040_VR]; Uppsala University
Organisations
Identifiers
ORCID iD: ORCID iD iconorcid.org/0000-0002-2178-9413

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