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1.
BMC Pulm Med ; 24(1): 310, 2024 Jul 02.
Article in English | MEDLINE | ID: mdl-38956567

ABSTRACT

BACKGROUND: Myasthenia gravis (MG) is the most common paraneoplastic disorder associated with thymic neoplasms. MG can develop after thymectomy, and this condition is referred to post-thymectomy myasthenia gravis (PTMG). Diffuse panbronchiolitis (DPB), is a rare form of bronchiolitis and is largely restricted to East Asia, has been reported in association with thymic neoplasms. Only three cases of combined MG and DPB have been reported in the literature. CASE PRESENTATION: A 45-year-old Taiwanese woman presented to our hospital with productive cough, rhinorrhea, anosmia, ear fullness, shortness of breath, and weight loss. She had a history of thymoma, and she underwent thymectomy with adjuvant radiotherapy 7 years ago. Chest computed tomography scan revealed diffuse bronchitis and bronchiolitis. DPB was confirmed after video-assisted thoracoscopic surgery lung biopsy, and repeated sputum cultures grew Pseudomonas aeruginosa. She has been on long-term oral azithromycin therapy thereafter. Intravenous antipseudomonal antibiotics, inhaled amikacin, as well as oral levofloxacin were administered. Three months after DPB diagnosis, she developed ptosis, muscle weakness, and hypercapnia requiring the use of noninvasive positive pressure ventilation. MG was diagnosed based on the acetylcholine receptor antibody and repetitive stimulation test results. Her muscle weakness gradually improved after pyridostigmine and corticosteroid therapies. Oral corticosteroids could be tapered off ten months after the diagnosis of MG. She is currently maintained on azithromycin, pyridostigmine, and inhaled amikacin therapies, with intravenous antibiotics administered occasionally during hospitalizations for respiratory infections. CONCLUSIONS: To our knowledge, this might be the first case report of sequential development of DPB followed by PTMG. The coexistence of these two disorders poses a therapeutic challenge for balancing infection control for DPB and immunosuppressant therapies for MG.


Subject(s)
Bronchiolitis , Myasthenia Gravis , Thymectomy , Thymus Neoplasms , Humans , Female , Myasthenia Gravis/etiology , Middle Aged , Bronchiolitis/etiology , Thymectomy/adverse effects , Thymus Neoplasms/surgery , Thymus Neoplasms/complications , Tomography, X-Ray Computed , Haemophilus Infections/etiology , Haemophilus Infections/diagnosis , Thymoma/surgery , Anti-Bacterial Agents/therapeutic use , Taiwan
2.
Surg Endosc ; 37(5): 3321-3339, 2023 05.
Article in English | MEDLINE | ID: mdl-36539629

ABSTRACT

OBJECTIVE: Thymectomy has been utilized as a treatment for Myasthenia Gravis (MG) for many decades, with both open and minimally invasive surgical (MIS) techniques currently used. Although MIS has shown improved short-term results, long-term effects remain uncertain. This systematic review and meta-analysis aim to compare the post-operative and long-term outcomes of MIS versus open thymectomy in MG patients. METHODS: MEDLINE, EMBASE and CENTRAL databases were searched from inception till January 2022 for keywords related to MG and open or MIS thymectomy. Primary outcome was complete stable remission (CSR), and secondary outcomes were clinical improvement, complications, length of stay, operation time, and blood loss. Grading of recommendations, assessment, development, and evaluation was used to assess the certainty of evidence. RESULTS: 26 studies with 3588 patients were included in the analysis. At 1, 3 and 5 years, there was no statistical difference noted in CSR between open versus MIS thymectomy. However, CSR was improved at 1 year for MIS thymectomy in non-thymomatous MG (P = 0.03). There was no significant difference in rates of partial clinical improvement between techniques at 1-year. Although analyses on length of hospital stay and blood loss showed improvement following MIS thymectomy, operative time was shorter for open thymectomy. CONCLUSION: This is the first systematic review and meta-analysis assessing long-term effects of MIS versus open thymectomy in MG patients. Given the lack of significant differences noted, either MIS or open thymectomy can be performed, based on surgeon preference. Further high-level, long-term research should be conducted to determine the benefit of each technique.


Subject(s)
Myasthenia Gravis , Thymectomy , Humans , Thymectomy/methods , Myasthenia Gravis/surgery , Myasthenia Gravis/etiology , Remission Induction , Length of Stay , Minimally Invasive Surgical Procedures , Treatment Outcome , Retrospective Studies
3.
Proc Natl Acad Sci U S A ; 117(48): 30649-30660, 2020 12 01.
Article in English | MEDLINE | ID: mdl-33199596

ABSTRACT

Myasthenia gravis (MG) is a neuromuscular, autoimmune disease caused by autoantibodies that target postsynaptic proteins, primarily the acetylcholine receptor (AChR) and inhibit signaling at the neuromuscular junction. The majority of patients under 50 y with AChR autoantibody MG have thymic lymphofollicular hyperplasia. The MG thymus is a reservoir of plasma cells that secrete disease-causing AChR autoantibodies and although thymectomy improves clinical scores, many patients fail to achieve complete stable remission without additional immunosuppressive treatments. We speculate that thymus-associated B cells and plasma cells persist in the circulation after thymectomy and that their persistence could explain incomplete responses to resection. We studied patients enrolled in a randomized clinical trial and used complementary modalities of B cell repertoire sequencing to characterize the thymus B cell repertoire and identify B cell clones that resided in the thymus and circulation before and 12 mo after thymectomy. Thymus-associated B cell clones were detected in the circulation by both mRNA-based and genomic DNA-based sequencing. These antigen-experienced B cells persisted in the circulation after thymectomy. Many circulating thymus-associated B cell clones were inferred to have originated and initially matured in the thymus before emigration from the thymus to the circulation. The persistence of thymus-associated B cells correlated with less favorable changes in clinical symptom measures, steroid dose required to manage symptoms, and marginal changes in AChR autoantibody titer. This investigation indicates that the diminished clinical response to thymectomy is related to persistent circulating thymus-associated B cell clones.


Subject(s)
B-Lymphocytes/metabolism , Lymphocyte Count , Myasthenia Gravis/blood , Thymus Gland/metabolism , Adolescent , Adult , Autoantibodies/immunology , B-Lymphocytes/immunology , Biomarkers , Clonal Evolution/genetics , Clonal Selection, Antigen-Mediated , Disease Susceptibility , Female , Humans , Male , Middle Aged , Models, Biological , Myasthenia Gravis/etiology , Radioimmunoassay , Receptors, Cholinergic/immunology , Thymectomy , Thymus Gland/cytology , Thymus Gland/immunology , V(D)J Recombination , Young Adult
4.
Acta Neurol Taiwan ; 32(4): 218-222, 2023 Dec 30.
Article in English | MEDLINE | ID: mdl-37723915

ABSTRACT

PURPOSE: Myasthenia gravis (MG) caused by COVID-19 vaccine had been reported, but the clinical course of new-onset ocular MG had never been described. We would like to document the clinical course of a patient with new-onset ocular MG which was caused by Pfizer-BioNTech COVID-19 vaccine. CASE REPORT: A 39-year-old woman noticed diplopia one week after she accepted the first dose of Pfizer- BioNTech COVID-19 vaccine. Diagnosis of ocular MG was made after investigation. Despite intravenous immunoglobulins, pyridostigmine and prednisolone therapy, she had no improvement until 10 days after treatment. She then rapidly improved, and almost fully recovered in the following 10 days. We had observed this patient for 8 months. After tapering off steroid, she remained stable to date, though she still suffered from transient diplopia on awakening. CONCLUSION: No matter the symptoms at onset, the clinical course or the response to steroid therapy was identical to ocular MG that we had ever known. Ocular MG caused by COVID-19 vaccine could probably be an iatrogenic life-long disease.


Subject(s)
COVID-19 , Myasthenia Gravis , Female , Humans , Adult , BNT162 Vaccine , COVID-19 Vaccines/adverse effects , Diplopia/etiology , COVID-19/prevention & control , Myasthenia Gravis/drug therapy , Myasthenia Gravis/etiology , Disease Progression
5.
J Korean Med Sci ; 37(10): e50, 2022 Mar 14.
Article in English | MEDLINE | ID: mdl-35289135

ABSTRACT

As coronavirus disease 2019 (COVID-19) has spread worldwide, the rate of COVID-19 vaccination uptake is encouraging. Neurological complications associated with COVID-19 vaccines such as stroke, Guillain-Barré syndrome, and Bell's palsy have been reported. Recently, late-onset myasthenia gravis (MG) following COVID-19 vaccination has been reported. To date, however, there has been no evidence of increased risk of early-onset MG following COVID-19. Here, we report a case of a patient with new-onset MG that arose after receiving a COVID-19 vaccine. A 33-year-old woman suddenly experienced generalized weakness and diplopia on the evening she had received the second dose of the Pfizer-BioNTech COVID-19 vaccine. The temporal relationship suggests that this new-onset MG is related to the vaccination. It also implies that COVID-19 vaccination could trigger early-onset MG symptoms in patients at risk of MG.


Subject(s)
BNT162 Vaccine/adverse effects , Myasthenia Gravis/etiology , Adult , Electromyography , Female , Humans , Myasthenia Gravis/diagnosis , Myasthenia Gravis/physiopathology , Neostigmine/pharmacology , Republic of Korea , Time Factors
6.
Muscle Nerve ; 63(6): 868-873, 2021 06.
Article in English | MEDLINE | ID: mdl-33675078

ABSTRACT

INTRODUCTION: There is limited evidence regarding the impact of World Health Organization (WHO) subtype of thymoma on post-thymectomy outcome of thymoma-associated myasthenia gravis (TAMG). The objective was to determine if the pathological subtypes of thymoma were associated with post-thymectomy outcomes of myasthenia gravis (MG), in patients with TAMG. METHODS: We performed a retrospective study of consecutive patients with TAMG who attended the neuromuscular clinic between January 2018 and December 2019 with a minimum follow-up of 1 y after thymectomy. Outcome measures were MG Impairment Index (MGII), single-simple question (SSQ), Myasthenia Gravis Foundation of America post-intervention status (MGFA PIS) and non-responder MG status at last assessment. RESULTS: Ninety-five patients were included; mean age at onset was 48.1 ± 12.1 y; 54(56.8%) were females. Thirteen patients developed MG post-thymectomy. The most common thymoma was WHO type B2 in 39 (41.1%). Most patients (40, 42.1%) had Masaoka stage II thymoma. There was no association of thymoma subtypes or Masaoka stage of disease with age, gender, MG phenotype, serology, post-thymectomy onset, interval from onset to thymectomy, MGII, SSQ, MGFA PIS, or non-responder status. Associations were found between positive serology and lower MGII (11.1 ± 14.2 vs 23 ± 12.9, P = .050), thymic follicular hyperplasia (TFH) and higher SSQ (89.3 ± 11.7 vs 80.1 ± 20.2, P-.043), and lack of recurrence and higher SSQ (84.1 ± 18 vs 72.5 ± 20, P = .037). DISCUSSION: The WHO pathological subtype of thymoma did not correlate with MG outcomes. However, positive acetylcholine antibody serology, presence of TFH, and non-recurrence of thymoma predict a favorable outcome.


Subject(s)
Myasthenia Gravis/etiology , Thymoma/complications , Thymus Neoplasms/complications , Adult , Female , Humans , Male , Middle Aged , Myasthenia Gravis/pathology , Myasthenia Gravis/surgery , Retrospective Studies , Thymectomy , Thymoma/pathology , Thymoma/surgery , Thymus Neoplasms/pathology , Thymus Neoplasms/surgery , Treatment Outcome
7.
Curr Rheumatol Rep ; 23(8): 63, 2021 07 03.
Article in English | MEDLINE | ID: mdl-34216297

ABSTRACT

PURPOSE: Myositis as a rare manifestation of COVID-19 is only recently being reported. This review examines the current literature on COVID-19-induced myositis focusing on etiopathogenesis, clinical presentations, diagnostic practices, and therapeutic challenges with immunosuppression, and the difficulties experienced by rheumatologists in established myositis in the COVID-19 era. RECENT FINDINGS: COVID-19 is associated with a viral myositis attributable to direct myocyte invasion or induction of autoimmunity. COVID-19-induced myositis may be varied in presentation, from typical dermatomyositis to rhabdomyolysis, and a paraspinal affliction with back pain. It may or may not present with acute exponential elevations of enzyme markers such as creatine kinase (CK). Virus-mediated muscle inflammation is attributed to ACE2 (angiotensin-converting enzyme) receptor-mediated direct entry and affliction of muscle fibers, leading on to innate and adaptive immune activation. A greater recognition of the stark similarity between anti-MDA5-positive myositis with COVID-19 has thrown researchers into the alley of exploration - finding common etiopathogenic basis as well as therapeutic strategies. For patients with established myositis, chronic care was disrupted during the pandemic with several logistic challenges and treatment dilemmas leading to high flare rates. Teleconsultation bridged the gap while ushering in an era of patient-led care with the digital transition to tools of remote disease assessment. COVID-19 has brought along greater insight into unique manifestations of COVID-19-related myositis, ranging from direct virus-induced muscle disease to triggered autoimmunity and other etiopathogenic links to explore. A remarkable shift in the means of delivering chronic care has led patients and caregivers worldwide to embrace a virtual shift with teleconsultation and opened doorways to a new era of patient-led care.


Subject(s)
COVID-19/physiopathology , Myositis/physiopathology , Rhabdomyolysis/physiopathology , Adaptive Immunity/immunology , Angiotensin-Converting Enzyme 2/metabolism , Autoantibodies/immunology , Back Pain/etiology , COVID-19/complications , COVID-19/immunology , COVID-19/metabolism , Creatine Kinase/metabolism , Dermatomyositis/etiology , Dermatomyositis/immunology , Dermatomyositis/metabolism , Dermatomyositis/physiopathology , Humans , Immunity, Innate/immunology , Interferon-Induced Helicase, IFIH1/immunology , Myasthenia Gravis/etiology , Myasthenia Gravis/immunology , Myasthenia Gravis/metabolism , Myasthenia Gravis/physiopathology , Myositis/etiology , Myositis/immunology , Myositis/metabolism , Paraspinal Muscles/physiopathology , Receptors, Coronavirus/metabolism , Rhabdomyolysis/etiology , Rhabdomyolysis/immunology , Rhabdomyolysis/metabolism , SARS-CoV-2
8.
Lung ; 199(3): 273-280, 2021 06.
Article in English | MEDLINE | ID: mdl-33987703

ABSTRACT

PURPOSE: This study aimed to investigate the association between asthma and risk of myasthenia gravis (MG) using the method of systematic review and meta-analysis. METHODS: Potentially eligible studies were identified from Medline and EMBASE databases from inception to July 2020 using search strategy that comprised terms for "Asthma" and "Myasthenia Gravis". Eligible cohort study must consist of one cohort of individuals with asthma and another cohort of individuals without asthma. Then, the study must report relative risk (RR) with 95% confidence intervals (95% CIs) of incident MG between the groups. Eligible case-control studies must include cases with MG and controls without MG. Then, the study must explore their history of asthma. Odds ratio (OR) with 95% CIs of the association between asthma status and MG must be reported. Point estimates with standard errors were retrieved from each study and were combined together using the generic inverse variance method. RESULTS: A total of 6,835 articles were identified. After two rounds of independent review by five investigators, two cohort studies and three case-control studies met the eligibility criteria and were included into the meta-analysis. Pooled analysis showed that asthma was significantly associated with risk of MG with the pooled risk ratio of 1.38 (95% CI 1.02-1.86). Funnel plot was symmetric, which was not suggestive of publication bias. CONCLUSION: The current study found a significant association between asthma and increased risk of MG.


Subject(s)
Asthma/complications , Myasthenia Gravis/etiology , Risk Assessment/methods , Asthma/epidemiology , Global Health , Humans , Incidence , Myasthenia Gravis/epidemiology , Risk Factors
9.
J Autoimmun ; 106: 102337, 2020 01.
Article in English | MEDLINE | ID: mdl-31594658

ABSTRACT

Thymomas are associated with a very high risk of developing Myasthenia Gravis (MG). Our objectives were to identify histological and biological parameters to allow early diagnosis of thymoma patients susceptible to developing MG. We conducted a detailed retrospective analysis from a patient database, searching for differences between patients with thymoma-associated MG (MGT, n = 409) and thymoma without MG (TOMA, n = 111) in comparison with nonthymomatous MG patients (MG, n = 1246). We also performed multiplex and single molecule arrays to measure the serum levels of cytokines in these groups of patients and controls (n = 14-22). We identified a set of parameters associated with MG development in thymoma patients: 1) detection of anti-acetylcholine receptor (AChR) antibodies, 2) development of B1 or B2 thymoma subtypes, 3) presence of ectopic thymic germinal centers (GCs), 4) local invasiveness of thymoma, and 5) being a woman under 50 years old. Among these parameters, 58.8% of MGT patients displayed GCs with a positive correlation between the number of GCs and anti-AChR titers. By immunohistochemistry, we found thymic GCs in the adjacent tissues of thymomas encircled by high endothelial venules (HEVs) that could favor peripheral cell recruitment. We also clearly associated MG symptoms with higher IFN-γ, IL-1ß and sCD40L serum levels, specifically in MGT patients compared to TOMA patients. Altogether, these analyses allowed the clear identification of histological, in particular the presence of GCs, and biological parameters that would facilitate the evaluation of the probability of the MG outcome postoperatively in thymoma patients.


Subject(s)
Germinal Center/pathology , Myasthenia Gravis/etiology , Thymoma/complications , Thymus Neoplasms/complications , Adult , Autoantibodies/metabolism , CD40 Ligand/metabolism , Female , Germinal Center/metabolism , Humans , Interferon-gamma/metabolism , Interleukin-1beta/metabolism , Male , Middle Aged , Myasthenia Gravis/metabolism , Receptors, Cholinergic/metabolism , Retrospective Studies , Risk Factors , Thymoma/metabolism , Thymus Neoplasms/metabolism
10.
J Neurol Neurosurg Psychiatry ; 91(5): 526-532, 2020 05.
Article in English | MEDLINE | ID: mdl-32165373

ABSTRACT

OBJECTIVE: Direct inhibition of acetylcholine receptor (AChR) function by autoantibodies (Abs) is considered a rare pathogenic mechanism in myasthenia gravis (MG), but is usually studied on AChRs expressed in cell lines, rather than tightly clustered by the intracellular scaffolding protein, rapsyn, as at the intact neuromuscular junction. We hypothesised that clustered AChRs would provide a better target for investigating the functional effects of AChR-Abs. METHODS: Acetylcholine-induced currents were measured using whole-cell patch clamping and a fast perfusion system to assess fast (<2 min) functional effects of the serum samples. The sensitivity, specificity and rapidity of the system were first demonstrated by applying maternal AChR-Ab positive plasmas known to inhibit fetal AChR function in TE671 cells. Eleven previously untested AChR-Ab positive MG sera, 10 AChR-Ab negative MG sera and 5 healthy control sera were then applied to unclustered and rapsyn-clustered human adult AChRs in CN21 cells. RESULTS: The maternal AChR-Ab positive plasmas reduced fetal AChR currents, but not adult AChR currents, by >80% within 100 s. Only 2/11 AChR-Ab positive sera inhibited AChR currents in unclustered AChRs, but 6/11 AChR-Ab positive sera compared with none of the 10 AChR-Ab negative sera (p=0.0020) inhibited rapsyn-clustered AChR currents, and current inhibition by the AChR-Ab positive sera was greater when the AChRs were clustered (p=0.0385). None of the sera had detectable effects on desensitisation or recovery from desensitisation. CONCLUSION: These results show that antibodies can inhibit AChR function rapidly and demonstrate the importance of clustering in exploring pathogenic disease mechanisms of MG Abs.


Subject(s)
Autoantibodies/immunology , Muscle Proteins/immunology , Myasthenia Gravis/immunology , Receptors, Cholinergic/immunology , Adolescent , Adult , Aged , Bungarotoxins/pharmacology , Cell Line , Electrophysiological Phenomena , Female , Fluoxetine/pharmacology , Humans , Male , Microscopy, Fluorescence , Middle Aged , Myasthenia Gravis/etiology , Patch-Clamp Techniques , Receptors, Cholinergic/drug effects , Young Adult
11.
Int J Neurosci ; 130(11): 1178-1181, 2020 Nov.
Article in English | MEDLINE | ID: mdl-32075478

ABSTRACT

Purpose: Myasthenia gravis (MG) is an autoimmune disease and closely related to thymoma. Inflammatory myopathy may accompany with other autoimmune diseases. However, concurrence of inflammatory myopathy and MG is very rare. Necrotizing autoimmune myopathy (NAM), a rare form of inflammatory myopathy, is characterized by necrosis and regeneration of myocytes in proximal muscles without significant inflammation. The aim of the study was to report a rare case of NAM and concomitant thymoma-associated MG after thymectomy.Materials and methods/results: A 27-year-old female patient presented with muscle soreness and weakness in four limbs. Eyelid fatigue and neostigmine tests were negative, and no ptosis was found but the electromyographic examination (EMG) showed myogenic damage and a gradual decrease in the amplitude (20%) of EMG activities evoked by repetitive electrical stimulation. Antibodies against AChR and increased titer of creatine kinase were detected and plaque-like signals in both legs were found in magnetic resonance imaging. Myositis-related antibodies were negative but necrotic myocytes without inflammatory cell infiltration, and MHC-1 positive muscle fibers were found in muscle biopsy. Pathological examination confirmed anterior mediastinal B2 type thymoma. Five weeks after thymectomy, she started to show typical MG symptoms. No recurrence of thymoma was found but immunoassay showed a higher titer of AChR-Ab. Myositis-related antibodies negative necrotizing autoimmune myopathy (NAM) was reported to be associated with thymoma-associated MG.Conclusions: The patient showed symptoms related NAM but developed MG-related symptoms only after thymectomy. The mechanisms for the phenomena may be related to immune dysfunction associated with thymoma.


Subject(s)
Autoimmune Diseases , Myasthenia Gravis , Myositis , Thymoma , Thymus Neoplasms , Adult , Autoimmune Diseases/diagnosis , Autoimmune Diseases/etiology , Female , Humans , Myasthenia Gravis/diagnosis , Myasthenia Gravis/etiology , Myositis/diagnosis , Myositis/etiology , Necrosis/diagnosis , Necrosis/etiology , Thymectomy , Thymoma/complications , Thymoma/surgery , Thymus Neoplasms/complications , Thymus Neoplasms/surgery
12.
Med J Malaysia ; 75(3): 295-297, 2020 05.
Article in English | MEDLINE | ID: mdl-32467548

ABSTRACT

Recurrent pneumonia warrants a diligent work-up to identify the underlying cause that perpetuates the disease process. Insidious bulbar dysfunction is arguably the most devastating as it would be diagnosed late after significant pulmonary complications due to chronic micro-aspiration. Bulbar disorder should be considered as the potential aetiology of recurrent pulmonary infections in the young population after excluding immunodeficiency disorder and respiratory anatomical anomaly. This report illustrates a rare case of bulbar onset myasthenia gravis which manifested as focal bronchiolectasis due to recurrent undiagnosed aspiration pneumonia three years earlier. Absence of hallmark features of Myasthenia Gravis (MG) such as ptosis, opthalmoplegia and proximal muscle weakness contributed to the diagnostic delay and challenges in this case. The diagnosis was established with the collaboration of multidisciplinary teams. Subsequent correct therapeutic interventions resulted in remarkable recovery in functional status and prevented her from further aspiration in the long run.


Subject(s)
Delayed Diagnosis , Myasthenia Gravis/diagnosis , Myasthenia Gravis/drug therapy , Cholinesterase Inhibitors/therapeutic use , Female , Humans , Myasthenia Gravis/etiology , Young Adult
13.
Emerg Infect Dis ; 25(5): 968-971, 2019 05.
Article in English | MEDLINE | ID: mdl-31002066

ABSTRACT

We report the rapid development of a myasthenic crisis as the first-time manifestation of myasthenia gravis. The symptoms developed in the course of acute leptospirosis associated with a new sequence type of Leptospira interrogans. Antibiotic treatment led to rapid amelioration of myasthenia.


Subject(s)
Leptospira interrogans/classification , Leptospira interrogans/genetics , Leptospirosis/complications , Leptospirosis/microbiology , Thyroid Crisis/diagnosis , Thyroid Crisis/etiology , Adult , Austria , DNA, Bacterial , Humans , Male , Myasthenia Gravis/complications , Myasthenia Gravis/etiology , Phylogeny , Severity of Illness Index , Symptom Assessment
14.
Reprod Biomed Online ; 38(4): 621-633, 2019 Apr.
Article in English | MEDLINE | ID: mdl-30795976

ABSTRACT

As all the structures of the human eye are characterized by sex hormone receptors, this study tested the hypothesis that assisted reproductive technology (ART) treatment influences visual function and ocular morphology in women who have undergone ART treatment and children born as a result of ART treatment. A systematic literature search of all original articles published up to August 2018 was performed using the PubMed database, including all original studies available in the literature. Review articles, studies in which participants underwent mixed interventions (i.e. other than ART treatment), studies reporting data on ocular malformations in ART offspring, and studies written in languages other than English were excluded. All selected articles were analysed to assess the level of evidence according to the Oxford Centre for Evidence-Based Medicine 2011 guidelines, and the quality of evidence according to the Grading of Recommendations Assessment, Development and Evaluation system. Although sparse data suggest that ART treatment can influence visual function and ocular morphology in women who have undergone ART treatment and children born as a result of ART treatment, the available evidence is inconclusive given its low level and quality. More high-quality research is needed to assess the potential interaction between ART treatment and the eye.


Subject(s)
Eye/drug effects , Reproductive Techniques, Assisted/adverse effects , Vision, Ocular/drug effects , Adult , Child , Child, Preschool , Choroidal Neovascularization/etiology , Cornea/drug effects , Female , Follow-Up Studies , Humans , Infant , Infant, Newborn , Intraocular Pressure/drug effects , Male , Myasthenia Gravis/etiology , Pregnancy , Retina/drug effects , Retinal Detachment/etiology , Retinal Vein/drug effects , Retinoblastoma/etiology , Retinopathy of Prematurity/etiology
15.
Lupus ; 28(4): 465-474, 2019 Apr.
Article in English | MEDLINE | ID: mdl-30739544

ABSTRACT

BACKGROUND: Despite its potentially significant impact on disease outcome, peripheral nervous system involvement in systemic lupus erythematosus has received little attention. OBJECTIVE: The objective of this study was to assess the prevalence and clinical features of peripheral nervous system involvement in a large cohort of systemic lupus erythematosus patients. METHODS: The records of systemic lupus erythematosus patients examined at two tertiary referral centres over a period of 14 years (from 2000 to 2014) were analyzed. Peripheral nervous system events were ascertained according to the 1999 American College of Rheumatology case definitions and by using an attribution algorithm for neuropsychiatric events. Prevalence of peripheral nervous system in systemic lupus erythematosus and demographic, clinical and laboratory features were assessed. Patients with peripheral nervous system events were compared with a control group of systemic lupus erythematosus patients without peripheral nervous system involvement. RESULTS: In a retrospective cohort of 1224 patients, the overall prevalence of peripheral nervous system involvement was 6.9% (85 patients, 95% confidence interval 0.06-0.08), with 68% of peripheral nervous system events attributable to systemic lupus erythematosus. Polyneuropathy was the most common manifestation observed (38 events, 39.2%), followed by cranial neuropathy in 30 cases (30.9%) and 12 cases of single (12.4%) or multiple (eight events, 8.2%) mononeuritis. The average age of systemic lupus erythematosus onset was significantly higher in patients with peripheral nervous system events than in controls (mean ± standard deviation: 45.9 ± 14.8 vs. 37.1 ± 14.0) and they were more likely to have higher SLEDAI-2K and SLICC/ACR Damage Index scores, as well as hypertension and livedo reticularis. A subgroup analysis of events deemed to be systemic lupus erythematosus-related provided similar results. CONCLUSION: Peripheral nervous system manifestations are a potential complication of systemic lupus erythematosus. Careful neurological assessment should therefore be included in the diagnostic workup of patients with systemic lupus erythematosus, especially in those with later onset and greater damage and disease activity.


Subject(s)
Cranial Nerve Diseases/epidemiology , Lupus Erythematosus, Systemic/epidemiology , Mononeuropathies/epidemiology , Myasthenia Gravis/epidemiology , Polyneuropathies/epidemiology , Adult , Antipsychotic Agents/therapeutic use , Cranial Nerve Diseases/drug therapy , Cranial Nerve Diseases/etiology , Female , Hospitals, University , Humans , Italy/epidemiology , Lupus Erythematosus, Systemic/complications , Male , Middle Aged , Mononeuropathies/drug therapy , Mononeuropathies/etiology , Myasthenia Gravis/drug therapy , Myasthenia Gravis/etiology , Myasthenia Gravis/physiopathology , Peripheral Nervous System/physiopathology , Polyneuropathies/drug therapy , Polyneuropathies/etiology , Prevalence , Retrospective Studies , Risk Factors , Severity of Illness Index , Tertiary Care Centers , Treatment Outcome , Young Adult
16.
Cancer Control ; 26(1): 1073274819865281, 2019.
Article in English | MEDLINE | ID: mdl-31331197

ABSTRACT

The aim of the study was to evaluate the usefulness of computed tomography (CT) and magnetic resonance imaging (MRI) for differentiating thymoma from nonthymoma abnormalities in patients with myasthenia gravis (MG). A cross-sectional study of 53 patients with MG, who had undergone surgical thymectomy, was conducted at 103 Hospital (Hanoi, Vietnam) and Cho Ray Hospital (Ho Chi Minh City, Vietnam) during August 2014 and January 2017. The CT and MRI images of patients with MG were qualitatively and quantitatively (radiodensity and chemical shift ratio [CSR]) analyzed to determine and compare their ability to distinguish thymoma from nonthymoma abnormalities. Logistic regression was used to identify the association between imaging parameters (eg, CSR) and the thymoma status. The receiver operating curve (ROC) analysis was used to determine the differentiating ability of CSR and radiodensity. As results, of the 53 patients with MG, 33 were with thymoma and 20 were with nonthymoma abnormalities. At qualitative assessment, MRI had significantly higher accuracy than did CT in differentiating thymoma from nonthymoma abnormalities (94.3% vs 83%). At quantitative assessment, both the radiodensity and CSR were significantly higher for thymoma compared with nonthymoma groups (P < .001). The ROC analysis showed that CSR had significantly higher sensitivity (Se) and specificity (Sp) than radiodensity in discriminating between the 2 groups (CSR: Se 100%, Sp 95% vs radiodensity: Se 90.9%, Sp 70%). When combining both qualitative and quantitative parameters, MRI had even higher accuracy than did CT in thymoma diagnosis (P = .031). In conclusion, chemical shift MRI was more accurate than CT for differentiating thymoma from nonthymoma in patients with MG.


Subject(s)
Myasthenia Gravis/etiology , Thymoma/diagnosis , Thymus Gland/diagnostic imaging , Thymus Neoplasms/diagnosis , Adult , Cross-Sectional Studies , Diagnosis, Differential , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , ROC Curve , Thymectomy , Thymoma/complications , Thymoma/surgery , Thymus Gland/surgery , Thymus Neoplasms/complications , Thymus Neoplasms/surgery , Tomography, X-Ray Computed , Young Adult
17.
BMC Gastroenterol ; 19(1): 92, 2019 Jun 14.
Article in English | MEDLINE | ID: mdl-31200650

ABSTRACT

BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm that originates from follicular dendritic cells in lymphoid tissue while paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms. Pancreatic FDCS associated with PNP and myasthenia gravis (MG) is even rarer and highly malignant. We present the clinical data, pathological materials and computed tomography (CT) features of a rare case of this disease. CASE PRESENTATION: A 49-year-old woman presented with repeated ptosis of both eyelids, oral ulcers and erosions. Her laboratory results showed a slight elevation of CA125 and positivity of some autoimmune antibodies. CT revealed a round solid mass with central necrosis in the pancreatic tail. The solid component of the mass showed slight enhancement and serpentine feeding arteries in the arterial phase, moderate enhancement with a draining vein around the tumor in the portal venous phase and persistent enhancement in the delayed phase. Surgical resection was performed, and the pathological diagnosis was FDCS. However, the patient died of inability to excrete sputum and occlusion of the respiratory tract. CONCLUSIONS: Pancreatic FDCS manifested as PNP and MG is very rare. Its CT features are not specific, and the disease should be differentiated from neuroendocrine tumors, solid pseudopapillary neoplasms and acinar cell carcinoma.


Subject(s)
Dendritic Cell Sarcoma, Follicular/diagnostic imaging , Myasthenia Gravis/diagnostic imaging , Pancreatic Neoplasms/diagnostic imaging , Paraneoplastic Syndromes/diagnostic imaging , Pemphigus/diagnostic imaging , Tomography, X-Ray Computed , Dendritic Cell Sarcoma, Follicular/complications , Female , Humans , Middle Aged , Myasthenia Gravis/etiology , Pancreas/diagnostic imaging , Pancreatic Neoplasms/complications , Paraneoplastic Syndromes/complications , Pemphigus/etiology
19.
BMC Neurol ; 18(1): 222, 2018 Dec 28.
Article in English | MEDLINE | ID: mdl-30593270

ABSTRACT

BACKGROUND: Myasthenia gravis (MG), an autoimmune neuromuscular disorder, occurs owing to autoantibodies against acetylcholine receptors. MG symptoms can be triggered by various vaccines. Many studies have evaluated the safety and adverse events of the human papillomavirus (HPV) vaccine. Here, we present a life-threatening case of ocular and bulbar MG symptoms after HPV vaccination and a brief literature review. CASE PRESENTATION: A 23-year-old woman presented with binocular diplopia, ptosis, dysarthria, and dysphagia, which occurred on the 3rd day after the second HPV vaccine administration. She was diagnosed with MG based on history, clinical features, and test results. Her symptoms deteriorated on the 3rd day after admission, and she was transferred to the intensive care unit with mechanical ventilation. On the 7th day after admission, due to discomfort in the right chest, pulmonary embolism was suspected. A tracheostomy was performed on the 14th day of mechanical ventilation. In the 4th week, the tracheostomy tube was removed; all symptoms had completely resolved at discharge. She was followed up for 5 months without recurrence or further treatment. CONCLUSION: HPV vaccination may cause MG owing to unexpected abnormal autoimmune responses. Additional studies are needed to clarify the possible causal relationship between the HPV vaccine and neurological complications and to evaluate the safety of the vaccine.


Subject(s)
Myasthenia Gravis/etiology , Papillomavirus Vaccines/adverse effects , Vaccination/adverse effects , Autoantibodies/immunology , Deglutition Disorders/etiology , Dysarthria/etiology , Female , Humans , Papillomavirus Vaccines/administration & dosage , Pulmonary Embolism/diagnosis , Receptors, Cholinergic/immunology , Young Adult
20.
BMC Neurol ; 18(1): 28, 2018 Mar 12.
Article in English | MEDLINE | ID: mdl-29529996

ABSTRACT

BACKGROUND: Case reports suggest there may be an association between celiac disease (CD) and myasthenia gravis (MG). METHODS: We identified 29,086 individuals with CD in Sweden from 1969 to 2008. We compared these individuals with 144,480 matched controls. Hazard ratios (HRs) for future MG (identified through ICD codes) were estimated using Cox regression. RESULTS: During 326,376 person-years of follow-up in CD patients, there were 7 MG cases (21/million person-years) compared to 22 MG cases in controls during 1,642,273 years of follow-up (14/million person-years) corresponding to a HR of 1.48 (95% CI = 0.64-3.41). HRs did not differ when stratifying for age, sex or calendar period. HRs were highest in the first year after follow-up, though insignificant. Individuals with CD were at no increased risk of MG more than 5 years after CD diagnosis (HR = 0.70; 95% CI = 0.16-3.09). CONCLUSION: This study found no increased risk of MG in patients with CD.


Subject(s)
Celiac Disease/complications , Myasthenia Gravis/epidemiology , Adolescent , Adult , Aged , Child , Female , Humans , Male , Middle Aged , Myasthenia Gravis/etiology , Proportional Hazards Models , Risk , Sweden/epidemiology , Young Adult
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