Database : MEDLINE
Search on : myasthenia and gravis [Words]
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[PMID]: 29524335
[Au] Autor:Yeo CJJ; Pleitez MY
[Ad] Address:Houston Methodist Neurological Institute, Houston Methodist Hospital, Texas Medical Center, 6565 Fannin Street, Houston, Texas, 77030.
[Ti] Title:Eculizumab in refractory myasthenic crisis.
[So] Source:Muscle Nerve;, 2018 Mar 10.
[Is] ISSN:1097-4598
[Cp] Country of publication:United States
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180310
[Lr] Last revision date:180310
[St] Status:Publisher
[do] DOI:10.1002/mus.26124

  2 / 15580 MEDLINE  
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[PMID]: 29524073
[Au] Autor:Dhillon S
[Ad] Address:Springer, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand. demail@springer.com.
[Ti] Title:Correction to: Eculizumab: A Review in Generalized Myasthenia Gravis.
[So] Source:Drugs;, 2018 Mar 09.
[Is] ISSN:1179-1950
[Cp] Country of publication:New Zealand
[La] Language:eng
[Ab] Abstract:The article Eculizumab: A Review in Generalized Myasthenia Gravis, written by Sohita Dhillon, was originally published Online First without open access.
[Pt] Publication type:PUBLISHED ERRATUM
[Em] Entry month:1803
[Cu] Class update date: 180310
[Lr] Last revision date:180310
[St] Status:Publisher
[do] DOI:10.1007/s40265-018-0889-3

  3 / 15580 MEDLINE  
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[PMID]: 29523340
[Au] Autor:Rodríguez Prida J; Trapiella Martínez L; Astudillo González A
[Ad] Address:Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Hospital Universitario de Cabueñes, Gijón, Asturias, España. Electronic address: xurdeprida@gmail.com.
[Ti] Title:Miositis de células gigantes asociada a miastenia gravis y timoma. Giant cell myositis associated with myasthenia gravis and thymoma.
[So] Source:Med Clin (Barc);, 2018 Mar 06.
[Is] ISSN:1578-8989
[Cp] Country of publication:Spain
[La] Language:eng; spa
[Pt] Publication type:LETTER
[Em] Entry month:1803
[Cu] Class update date: 180310
[Lr] Last revision date:180310
[St] Status:Publisher

  4 / 15580 MEDLINE  
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[PMID]: 29522809
[Au] Autor:Mineo TC; Tamburrini A; Schillaci O; Ambrogi V
[Ad] Address:Department of Surgery and Experimental Medicine, Multidisciplinary Myasthenia Gravis Unit, Policlinico Tor Vergata University, Rome, Italy. Electronic address: mineo@med.uniroma2.it.
[Ti] Title:Onset and Evolution of Clinically Apparent Myasthenia Gravis after Resection of Non-Myasthenic Thymomas.
[So] Source:Semin Thorac Cardiovasc Surg;, 2018 Mar 06.
[Is] ISSN:1532-9488
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: Patients with thymoma and without clinical or electromyographical myasthenic signs may occasionally develop myasthenia several years after thymectomy. Hereby, we investigated the predictors and the evolution of this peculiar disease. METHODS: We performed a retrospective analysis in 104 consecutive patients undergoing thymectomy between 1987 and 2013 for thymoma without clinical nor electromyographic signs of myasthenia gravis. Predictors of post-thymectomy onset of myasthenia gravis were investigated with univariale time-to-disease analysis. Evolution of myasthenia was analyzed with time-to-regression analysis. RESULTS: Eight patients developed late myasthenia gravis after a median period of 33 months from thymectomy. No significant correlation was found for age, gender, Masaoka's stage and World Health Organization histology. Only high preoperative serum acetylcholine-receptor antibodies titer (>0.3 nmol/L) was significantly associated with post-thymectomy myasthenia gravis at univariale time-to-disease (p=0.003) analysis. Positron emission tomography was always performed in high-titer patients detecting activity in 4 of these. Surgical treatment through redo-sternotomy or video-thoracoscopy was performed in these last cases with a remission in all patients after 12, 24, 32 and 48 months, respectively. No patient under medical treatment has yet developed a complete remission. CONCLUSIONS: In our study the presence of pre-operative high-level serum acetylcholine receptor antibodies was the only factor significantly associated with the development of post-thymectomy myasthenia gravis. The persistence of residual islet of ectopic thymic tissue was one of the causes of the onset of myasthenia and surgical removal of these achieved satisfactory results.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher

  5 / 15580 MEDLINE  
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[PMID]: 29522637
[Au] Autor:Mora M; Saluto V; Balbi P; Spotti M; Fadel D
[Ad] Address:Servicio de Salud Mental Instituto de Investigaciones Médicas "A. Lanari", Facultad de Medicina, UBA.
[Ti] Title:Síndrome de Munchausen mixto con comorbilidad orgánica. [Mixed Munchausen Syndrome with organic comorbidity].
[So] Source:Vertex;XXVIII(132):145-151, 2017 Mar.
[Is] ISSN:0327-6139
[Cp] Country of publication:Argentina
[La] Language:spa
[Ab] Abstract:We present a detailed case report that shows a woman patient who has Factitious Disorder manifested by the coexistence both of: A) typical/direct Munchausen and B) Munchausen by proxy or indirect: being the frst one (A) about the own person and the second one (B) about other people (most cases about their own young children). Furthermore, in the reported case we observed that the patient shown the particularity of having positive biological markers for Myasthenia Gravis (serology markers), and having inconsistent clinical manifestations that are typically observed in the exacerbation phase when she still continued in remission phase. In our own bibliographic research we couldn`t fnd anything about this case of "Mixed Munchausen Syndrome with organic comorbidity". In the same way as we tried to get information about the diagnostic algorithms and the possible therapeutic treatment strategies we found nothing like this reported before. Finally, this clinical presentation constitutes a blind spot for the scientifc community generating a lack of recognition for this diagnostic category and above all of the confusion that this mental disorder can generate in: a) the inadequate use of therapeutic resources, b) the irrational use of drugs, c) the distortion of institutional instances, and d) the medical behaviors that occurred in this case.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:In-Data-Review

  6 / 15580 MEDLINE  
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[PMID]: 29435915
[Au] Autor:Dhillon S
[Ad] Address:Springer, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand. demail@springer.com.
[Ti] Title:Eculizumab: A Review in Generalized Myasthenia Gravis.
[So] Source:Drugs;78(3):367-376, 2018 Mar.
[Is] ISSN:1179-1950
[Cp] Country of publication:New Zealand
[La] Language:eng
[Ab] Abstract:The humanized monoclonal antibody eculizumab (Soliris ) is a complement inhibitor indicated for use in anti-acetylcholine receptor (AChR) antibody-positive adults with generalized myasthenia gravis (gMG) in the USA, refractory gMG in the EU, or gMG with symptoms that are difficult to control with high-dose IVIg therapy or PLEX in Japan. It is the first complement inhibitor to be approved for use in these patients. In the well-designed, 26-week REGAIN study in patients with anti-AChR-positive refractory gMG, although a statistically significant benefit of eculizumab over placebo in the prespecified primary endpoint analysis (change from baseline in MG-activities of daily living (ADL) score assessed by worst-rank ANCOVA) was not formally demonstrated, preplanned and post hoc sensitivity analyses of this outcome, as well as other secondary outcomes supported the efficacy of eculizumab. Overall, patients receiving eculizumab experienced significant improvements in the ADL, muscle strength and health-related quality of life (HR-QOL) parameters relative to patients receiving placebo. Moreover, an ongoing extension of REGAIN showed that treatment benefits with eculizumab were sustained during continued therapy for at least 52 weeks. Eculizumab was generally well tolerated in these studies, with a tolerability profile similar to that reported previously in other indications. Although several questions remain, such as duration of treatment, cost effectiveness and long-term efficacy and tolerability, current evidence indicates that eculizumab is a valuable emerging therapy for patients with refractory gMG.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180310
[Lr] Last revision date:180310
[St] Status:In-Process
[do] DOI:10.1007/s40265-018-0875-9

  7 / 15580 MEDLINE  
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[PMID]: 29518096
[Au] Autor:Park KH; Waters P; Woodhall M; Lang B; Smith T; Sung JJ; Kim KK; Lim YM; Kim JE; Kim BJ; Park JS; Lim JG; Kim DS; Kwon O; Sohn EH; Bae JS; Yoon BN; Kim NH; Ahn SW; Oh J; Park HJ; Shin KJ; Hong YH
[Ad] Address:Department of Neurology, Gyeongsang National University Hospital, Jinju, Republic of Korea.
[Ti] Title:Myasthenia gravis seronegative for acetylcholine receptor antibodies in South Korea: Autoantibody profiles and clinical features.
[So] Source:PLoS One;13(3):e0193723, 2018.
[Is] ISSN:1932-6203
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Acquired myasthenia gravis (MG) is a prototype autoimmune disease of the neuromuscular junction, caused in most patients by autoantibodies to the muscle nicotinic acetylcholine receptor (AChR). There seem to be ethnic and regional differences in the frequency and clinical features of MG seronegative for the AChR antibody. This study aimed to describe the autoantibody profiles and clinical features of Korean patients with generalized MG seronegative for the AChR antibody. A total of 62 patients with a high index of clinical suspicion of seronegative generalized MG were identified from 18 centers, and we examined their sera for antibodies to clustered AChR, muscle-specific tyrosine kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (LRP4) by cell-based assays (CBA) and to MuSK by radioimmunoprecipitation assay (RIPA). We also included 8 patients with ocular MG, 3 with Lambert-Eaton myasthenic syndrome, 5 with motor neuron disease, and 9 with other diagnoses as comparators for the serological testing. Antibodies were identified in 25/62 (40.3%) patients: 7 had antibodies to clustered AChR, 17 to MuSK, and 2 to LRP4. Three patients were double seropositive: 1 for MuSK and LRP4, and 2 for MuSK and clustered AChR. The patients with MuSK antibodies were mostly female (88.2%) and characterized by predominantly bulbar involvement (70%) and frequent myasthenic crises (58.3%). The patients with antibodies to clustered AChR, including 2 with ocular MG, tended to have a mild phenotype and good prognosis.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:In-Data-Review
[do] DOI:10.1371/journal.pone.0193723

  8 / 15580 MEDLINE  
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[PMID]: 29205338
[Au] Autor:Pelz A; Schaffert H; Diallo R; Hiepe F; Meisel A; Kohler S
[Ad] Address:Department of Experimental Neurology, Charité - Universitätsmedizin Berlin, Berlin, Germany.
[Ti] Title:S1P receptor antagonists fingolimod and siponimod do not improve the outcome of experimental autoimmune myasthenia gravis mice after disease onset.
[So] Source:Eur J Immunol;48(3):498-508, 2018 Mar.
[Is] ISSN:1521-4141
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:Myasthenia gravis (MG) is an autoimmune disease characterized by muscle weakness and fatigue in the presence of circulating antibodies against components of the neuromuscular junction. Most patients have a good prognosis, but some are refractory to standard-of-care immunosuppressive treatment and suffer from recurrent myasthenic crises. Functional sphingosine-1-phosphate (S1P) antagonists like fingolimod and siponimod (BAF312) are successfully used for the treatment of multiple sclerosis, and fingolimod was shown to prevent the development of myasthenic symptoms in experimental autoimmune myasthenia gravis (EAMG), the standard model of MG. Here, we investigated whether fingolimod or siponimod improves outcome in EAMG mice when administered after disease onset, modeling the clinical setting in human MG. Both S1P antagonists inhibited lymphocyte egress, resulting in peripheral lymphopenia. After stimulation, there were differences in T-cell responses, but no change in either antibody titers or total or antigen-specific plasma cell populations after treatment. Most importantly, disease incidence and severity were not influenced by fingolimod or siponimod therapy. Although fingolimod and siponimod did lead to subtle changes in T-cell responses, they had no significant effect on antibody titers and disease severity. In conclusion, our data show no evidence of a therapeutic potential for S1P receptor antagonists in MG treatment.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:In-Data-Review
[do] DOI:10.1002/eji.201747187

  9 / 15580 MEDLINE  
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[PMID]: 29515082
[Au] Autor:Onuki T; Ueda S; Otsu S; Yanagihara T; Kawakami N; Yamaoka M; Inagaki M
[Ad] Address:Department of General Thoracic Surgery, Tsuchiura Kyodo General Hospital, Tsuchiura, Ibaraki, Japan.
[Ti] Title:Thymectomy during Myasthenic Crisis under Artificial Respiration.
[So] Source:Ann Thorac Cardiovasc Surg;, 2018 Mar 07.
[Is] ISSN:2186-1005
[Cp] Country of publication:Japan
[La] Language:eng
[Ab] Abstract:A 34-year-old man was diagnosed with thymoma, which was evaluated preoperatively as stage II or III, with myasthenia gravis (MG). The size of the tumor was 70 × 44 × 80 mm. No invasion to neighboring organs was observed. Prednisolone was prescribed for stabilization of MG. However, a myasthenic crisis (MC) occurred, and intensive care, including emergent endobronchial intubation followed by artificial ventilation, pulse steroid therapy, high-dose intravenous immunoglobulin, and tacrolimus hydrate, was initiated. A chest computed tomography on day 6 revealed tumor reduction to 50 × 30 × 60 mm. An extended total thymectomy by median sternotomy was performed, and artificial ventilation was continued after that. Scheduled artificial ventilation and steroid therapy together can, therefore, enable complete resection of thymoma in patients undergoing treatment for MC. While ventilation helps avert a respiratory failure, the steroid therapy temporarily reduces the tumor size, making resection easier.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:Publisher
[do] DOI:10.5761/atcs.cr.17-00176

  10 / 15580 MEDLINE  
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[PMID]: 29293936
[Au] Autor:Rao AK; Lin NH; Jackson KA; Mody RK; Griffin PM
[Ad] Address:Enteric Diseases Epidemiology Branch, Division of Foodborne, Waterborne, and Environmental Diseases, National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Georgia.
[Ti] Title:Clinical Characteristics and Ancillary Test Results Among Patients With Botulism-United States, 2002-2015.
[So] Source:Clin Infect Dis;66(suppl_1):S4-S10, 2017 Dec 27.
[Is] ISSN:1537-6591
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Background: Botulism is classically described as a bilateral, symmetric, descending flaccid paralysis in an afebrile and alert patient without sensory findings. We describe the reported spectrum of clinical findings among persons >12 months of age in the United States during 2002-2015. Methods: The Centers for Disease Control and Prevention collects clinical findings reported by physicians treating suspected cases of botulism nationwide. We analyzed symptoms and signs, and neuroimaging and cerebrospinal fluid (CSF) results. A case was defined as illness compatible with botulism with laboratory confirmation or epidemiologic link to a confirmed case, and presence or absence of at least 1 sign or symptom recorded. Physicians' differential diagnoses were evaluated. Results: Clinical information was evaluated for 332 botulism cases; data quality and completeness were variable. Most had no fever (99%), descending paralysis (93%), no mental status change (91%), at least 1 ocular weakness finding (84%), and neuroimaging without acute changes (82%). Some had paresthesias (17%), elevated CSF protein level (13%), and other features sometimes considered indicative of alternative diagnoses. Five of 71 (7%) cases with sufficient information were reported to have atypical findings (eg, at least 1 cranial nerve finding that was unilateral or ascending paralysis). Illnesses on the physician differential included Guillain-Barré syndrome (99 cases) and myasthenia gravis (76 cases) and, rarely, gastrointestinal-related illness (5 cases), multiple sclerosis (3 cases), sepsis (3 cases), and Lyme disease (2 cases). Conclusions: Our analysis illustrates that classic symptoms and signs were common among patients with botulism but that features considered atypical were reported by some physicians. Diagnosis can be challenging, as illustrated by the broad range of illnesses on physician differentials.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:In-Data-Review
[do] DOI:10.1093/cid/cix935


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