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Neurotherapeutics 2022 May 12. Epub 2022 May 12.

Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.

In recent years, there has been increasing recognition of the diversity of autoimmune neurological diseases affecting all levels of the nervous system. A growing understanding of disease pathogenesis has enabled us to better target specific elements of the immune system responsible for the cell dysfunction and cell destruction seen in these diseases. This is no better demonstrated than in the development of complement directed therapies for the treatment of complement mediated autoimmune neurological conditions. Read More

J Cardiothorac Surg 2022 May 12;17(1):115. Epub 2022 May 12.

Department of Cardiothoracic Surgery, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China.

Background: Post-thymectomy myasthenia gravis (PTMG) is defined as thymoma patients without signs of myasthenia gravis (MG) pre-operation, but develop MG after radical surgical resection. PTMG might be misdiagnosed not only because of its rare incidence, but also the uncertain interval between the removal of thymoma and the new onset MG. Additionally, some surgeons and anesthesiologists pay less attention to those asymptomatic thymoma patients in perioperative management, leading to the neglect of new onset PTMG, and miss the best time to treat it. Read More

J Innate Immun 2022 May 12:1-21. Epub 2022 May 12.

Department of Clinical Genetics, LUMC, Leiden, The Netherlands.

Damage and disease of nerves activates the complement system. We demonstrated that activation of the terminal pathway of the complement system leads to the formation of the membrane attack complex (MAC) and delays regeneration in the peripheral nervous system. Animals deficient in the complement component C6 showed improved recovery after neuronal trauma. Read More

Rev Neurol 2022 May;74(10):343-344

Complejo Hospitalario Universitario de Albacete, Albacete, España.

Front Neurol 2022 25;13:857402. Epub 2022 Apr 25.

Department of Neurology, Tangdu Hospital, Air Force Medical University, Xi'an, China.

During the past two decades, an increasing number of patients with very-late-onset myasthenia gravis (v-LOMG) with an onset age of 65 years or older have been identified. However, few studies explore the predictors of secondary generalization in patients with v-LOMG with pure ocular onset. In this retrospective cohort study, 69 patients with v-LOMG were divided into ocular MG (OMG) and generalized MG (GMG), and the clinical characteristics and outcomes were compared. Read More

Front Neurol 2022 25;13:886625. Epub 2022 Apr 25.

Department of Neurology and Rehabilitation Medicine, George Washington University, Washington, DC, United States.

Chronic, high-dose, oral prednisone has been the mainstay of myasthenia gravis treatment for decades and has proven to be highly beneficial in many, toxic in some way to all, and not effective in a significant minority. No patient characteristics or biomarkers are predictive of treatment response leading to many patients suffering adverse effects with no benefit. Presently, measurements of treatment response, whether taken from clinician or patient perspective, are appreciated to be limited by lack of good correlation, which then complicates correlation to biological measures. Read More

BMJ Case Rep 2022 May 11;15(5). Epub 2022 May 11.

Clin Shoulder Elb 2022 May 11. Epub 2022 May 11.

Department of Orthopaedic Surgery, Konkuk University School of Medicine, Seoul, Korea.

The authors present a case of transient postoperative inferior subluxation of the shoulder after arthroscopic surgical stabilization for recurrent anterior dislocation. The patient was a 61-year-old woman with myasthenia gravis (MG). The first anterior shoulder dislocation occurred because of a fall to the ground. Read More

Neuropsychiatr Dis Treat 2022 3;18:953-964. Epub 2022 May 3.

Department of Neurology, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jinan, Shandong, People's Republic of China.

Purpose: To evaluate the efficacy and safety of low dosages of rituximab (RTX) in the treatment of MuSK-antibody-positive MG patients.

Patients And Methods: We retrospectively analyzed the data of MuSK-antibody-positive MG patients who were treated with low dosages of RTX from January 2018 to October 2021. The long-term treatment response to RTX was assessed by Myasthenia Gravis Foundation of America (MGFA) post-interventional status (PIS), Myasthenia Gravis Status and Treatment Intensity (MGSTI), dosage of steroid, MG-related activities of daily living (MG-ADL) and myasthenic muscle score (MMS) at the end of follow-up. Read More

BMC Neurol 2022 May 9;22(1):172. Epub 2022 May 9.

Department of Neurology and Centre for Rare Diseases, Institute of Immunology and Imunotherapy, University Hospitals Birmingham and University of Birmingham, Mindelsohn Way, Edgbaston, Birmingham, B15 2GW, UK.

Background: Patients with generalized myasthenia gravis (MG) often experience debilitating exacerbations, with the possibility of life-threatening respiratory crises requiring hospitalization. Long-term longitudinal studies are needed to understand the burden of MG, including in patients whose disease is refractory to conventional treatment.

Methods: A retrospective, longitudinal, cohort study was conducted of patients in England aged ≥ 18 years with treatment-refractory or non-refractory MG, using data recorded during 1997-2016 in the Clinical Practice Research Datalink and the Hospital Episode Statistics databases. Read More

Pract Neurol 2022 May 9. Epub 2022 May 9.

Department of Neurology, Principe de Asturias University Hospital, Madrid, Spain.

Oculopharyngeal muscular dystrophy (OPMD) and myasthenia gravis are both rare conditions of different pathophysiological nature, so a fortuitous association is unlikely. However, antiacetylcholine receptor antibodies may be found in other genetic myopathies and this is probably not coincidental. Muscle fibre degeneration can induce innate immune responses that may break immune tolerance and allow the generation of autoantibodies to muscle proteins. Read More

Front Immunol 2022 22;13:867181. Epub 2022 Apr 22.

Department of Anatomical, Histological, Forensic and Orthopedic Sciences, Section of Histology and Medical Embryology, Sapienza University of Rome, Rome, Italy.

Thymic Epithelial Tumors (TETs) arise from epithelial cells of the thymus and are very rare neoplasms comprising Thymoma, Thymic carcinoma, and Thymic Neuroendocrine tumors that still require in-depth molecular characterization. Long non-coding RNAs (lncRNAs) are emerging as relevant gene expression modulators involved in the deregulation of several networks in almost all types of human cancer, including TETs. LncRNAs act at different control levels in the regulation of gene expression, from transcription to translation, and modulate several pathways relevant to cell fate determination under normal and pathological conditions. Read More

Neurol Sci 2022 May 8. Epub 2022 May 8.

Cerrahpasa Faculty of Medicine, Department of Neurology, Istanbul University-Cerrahpasa, Istanbul, Turkey.

Neurol Sci 2022 May 6. Epub 2022 May 6.

Peripheral Nervous System, Vall d'Hebron Institut de Recerca (VHIR), Vall d'Hebron Hospital Universitari, Vall d'Hebron Barcelona Hospital Campus, Passeig Vall d'Hebron 119-129, 08035, Barcelona, Spain.

Background: Myasthenia gravis (MG) is a very heterogenic chronic autoimmune disease caused by the failure of neuromuscular transmission. The HLA gene complex has conventionally been recognized as its main genetic risk and phenotype modifying factor. Our aim was to investigate the prevalence of HLA class I and II alleles and to identify possible risk factors for sporadic MG in a Spanish cohort. Read More

Front Immunol 2022 14;13:757841. Epub 2022 Apr 14.

Department of Neurology, Xiangya Hospital, Central South University, Changsha, China.

Background: Lymphoplasma exchange (LPE), a technique combining plasma exchange with leukapheresis, is emerging as promising treatment for autoimmune diseases. Data on the efficacy and safety of LPE in myasthenia gravis (MG) therapy are scarce. In this study, we aimed to comprehensively review the clinical efficacy, safety, and immunological characteristics of LPE therapy in MG patients. Read More

Neurol Ther 2022 May 5. Epub 2022 May 5.

Service Système Nerveux Périphérique et Muscles, Centre Hospitalier Universitaire de Nice, Université Côte d'Azur, Nice, France.

Introduction: Lambert-Eaton myasthenic syndrome (LEMS) is characterized by autoantibodies against voltage-gated calcium channels (VGCC) at the neuromuscular junction causing proximal muscle weakness, decreased tendon reflexes, and autonomic changes. The European LEMS registry aimed to collate observational safety data for 3,4-diaminopyridine phosphate (3,4-DAPP) and examine long-term outcomes for patients with LEMS.

Methods: Thirty centers across four countries participated in the non-interventional European LEMS registry. Read More

J Neurol 2022 May 3. Epub 2022 May 3.

Ca'foncello Hospital, Treviso, Italy.

Introduction: Myasthenia gravis (MG) is an autoimmune disease, for which the risk of exacerbation after vaccines is debated. The aim of this study is to review the available literature concerning safety and efficacy of vaccines in MG. In addition, we also conducted a retrospective research of MG exacerbations and new onset MG after anti-SARS-CoV-2 vaccination in a large cohort of patients. Read More

BMC Neurol 2022 May 3;22(1):168. Epub 2022 May 3.

Department of Neurology, The Second Xiangya Hospital, Central South University, Changsha, Hunan, China.

Background: HINT1 mutations cause an autosomal recessive axonal neuropathy with neuromyotonia. This is a first case report of coexistence of myasthenia gravis (MG) and HINT1-related motor axonal neuropathy without neuromyotonia.

Case Presentation: A 32-year-old woman presented with recurrent ptosis for 8 years, diplopia for 2 years and limb weakness for 1 year and a half. Read More

Neuromuscul Disord 2022 Mar 19. Epub 2022 Mar 19.

Department of Respiratory Diseases, Azienda Ospedaliero Universitaria, Modena, Italy.

J Vis Exp 2022 Apr 14(182). Epub 2022 Apr 14.

Department of Biomedical Engineering, Columbia University; Department of Medicine, Columbia University; College of Dental Medicine, Columbia University;

Many neuromuscular diseases, such as myasthenia gravis (MG), are associated with dysfunction of the neuromuscular junction (NMJ), which is difficult to characterize in animal models due to physiological differences between animals and humans. Tissue engineering offers opportunities to provide in vitro models of functional human NMJs that can be used to diagnose and investigate NMJ pathologies and test potential therapeutics. By incorporating optogenetic proteins into induced pluripotent stem cells (iPSCs), we generated neurons that can be stimulated with specific wavelengths of light. Read More

Orbit 2022 May 2:1-5. Epub 2022 May 2.

Department of Ophthalmology, The Royal Victorian Eye and Ear Hospital, Melbourne, Victoria,Australia.

Myasthenia gravis (MG) is an autoimmune disease that results in muscle weakness and fatigability. Extraocular involvement may be the first sign of disease. It may be triggered by infections, stress, or medications. Read More

Am J Case Rep 2022 May 2;23:e936000. Epub 2022 May 2.

Department of Neurology, Aichi Medical University, Nagakute, Aichi, Japan.

BACKGROUND Myasthenia gravis (MG) is an autoimmune neuromuscular disorder, which is often accompanied by various complications. Partial dysgeusia is an uncommon nonmotor symptom of MG, and dysgeusia preceding typical MG symptoms is rare. Although ageusia and hypogeusia have been reported in patients with MG, increased perception of taste has not been reported. Read More

Semin Arthritis Rheum 2022 Apr 15;55:152010. Epub 2022 Apr 15.

Harvard Medical School, Massachusetts General Hospital, Boston, MA, United States.

Glucocorticoids (GCs) have been the cornerstone of treating dozens of inflammatory conditions for more than seven decades. GC toxicity is ubiquitous in both clinical trials and clinical practice, and toxicities associated with GC use are central to the experience of most patients being treated for immune-mediated conditions. These conditions span the full range of medical specialties, including rheumatology, nephrology, gastroenterology, neurology, pulmonology, ophthalmology, and others. Read More

J Neurol Sci 2022 Apr 21;437:120268. Epub 2022 Apr 21.

Evidera, 500 Totten Pond Road, Waltham, MA, USA. Electronic address:

Background/objectives: While the clinical manifestations of myasthenia gravis (MG) are well understood, its humanistic impact is not. The objective of this systematic literature review (SLR) was to provide a comprehensive understanding of the humanistic burden of MG with regards to psychological symptoms and health-related quality of life (HRQoL) according to patients and caregivers.

Methods: A systematic search was conducted on December 27, 2019, in MEDLINE and Embase to identify English-language studies that were published from January 1, 2009-December 27, 2019 and presented relevant information on the humanistic burden among adults with MG and their caregivers. Read More

Pediatr Blood Cancer 2022 Apr 29:e29723. Epub 2022 Apr 29.

Division of Rheumatology, Department of Pediatrics, Perelman School of Medicine, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

Neurol Sci 2022 Apr 28. Epub 2022 Apr 28.

Department of Neurology, Dongsan Medical Center, Keimyung University School of Medicine, 1035 Dalgubeol-daero, Dalseo-gu, Daegu, 42601, Republic of Korea.

Pak J Med Sci 2022 Mar-Apr;38(3Part-I):530-535

Ying Yang, Center of Treatment of Myasthenia Gravis Hebei Province, Shi Jiazhuang People's Hospital, Shi Jiazhuang, 050000, Hebei, China.

Objectives: To investigate the clinical features of patients with myasthenia gravis complicated with and without hyperthyroidism.

Methods: A total of 2083 patients with myasthenia gravis (MG) admitted in Center of Treatment of Myasthenia Gravis Hebei Province between January 2013 and July 2020 were retrospectively analyzed and divided into two groups: Group-A and Group-B, with 108 MG patients complicated with hyperthyroidism in Group-A and 1975 MG patients without thyroid disease in Group-B. The age of onset, gender, Osserman classification, acetylcholine receptor antibody and thymus status of the two groups were analyzed in the two groups. Read More

JA Clin Rep 2022 Apr 28;8(1):33. Epub 2022 Apr 28.

Department of Anesthesiology, Saiseikai Fukuoka General Hospital, Tenjin 1-3-46, Chuo-ku, Fukuoka-shi, Fukuoka-ken, 810-0001, Japan.

Background: First-degree atrioventricular block (AVB) may lead to complete AVB. Herein, we present a case of a complete AVB under thoracic epidural catheter infusion of ropivacaine with fentanyl in a patient with first-degree AVB and myasthenia gravis.

Case Presentation: A 74-year-old woman with first-degree AVB underwent thymectomy for myasthenia gravis. Read More

Trials 2022 Apr 27;23(1):365. Epub 2022 Apr 27.

Department of Gastroenterology, The First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, China.

Background: Myasthenia gravis (MG) is an acquired autoimmune disease with high heterogeneity. The disease is chronic, relapsing repeatedly and progressive with acute exacerbation occasionally. Although the treatment of MG has developed, it is still unsatisfactory and has some unexpected side effects. Read More

Can J Neurol Sci 2022 Apr 28:1-8. Epub 2022 Apr 28.