- |||||||||| Journal: Mechanism of the Long-lasting Potentiating Effect of Distigmine on Urinary Bladder Motility (Pubmed Central) - May 21, 2021
We also discuss the long-lasting potentiating effect of distigmine on urinary bladder motility and the mechanism of these effects using guinea pig urinary bladder smooth muscle in vivo and in vitro. In addition, we present our investigations on the long-lasting mechanism of distigmine using recombinant human acetylcholinesterase.
- |||||||||| Journal: Impact of Coronavirus Disease 2019 in a French Cohort of Myasthenia Gravis. (Pubmed Central) - May 20, 2021
However, the risk of severe COVID-19 is elevated in patients with high MGFA classes [odds ratio: 102.6 (4.4; 2,371.9)]. These results are important for establishing evidence-based guidelines for the management of MG patients during the COVID-19 pandemic.
- |||||||||| [VIRTUAL] Impact of age-associated decreases in thymic B cell expression of Aire and self-antigen genes on T cell tolerance () - May 19, 2021 - Abstract #IMMUNOLOGY2021IMMUNOLOGY_1924;
We also used MHCII tetramers and ELISpot to detect T cells recognizing a model TRA expressed in thymic B cells, Titin (Ttn), an auto-antigen involved in late-onset myasthenia gravis, in the thymus and secondary lymphoid organs of young and aged mice. Together, our preliminary results suggest that the reduced capacity of aged thymic B cells to express Aire also impairs their ability to properly tolerize developing T cells in the thymus.
- |||||||||| Preclinical, Journal: Introducing a mammalian nerve-muscle preparation ideal for physiology and microscopy, the transverse auricular muscle in the ear of the mouse. (Pubmed Central) - May 15, 2021
These new observations strongly encourage further in-depth studies of ear-muscle architecture, in the many available mouse-models of various devastating human muscle-diseases. Finally, we demonstrate that the delicate and defined physical characteristics of this 'new' mammalian muscle are ideal for ultrastructural study, and thereby facilitate the imaging of synaptic vesicle membrane recycling in mammalian neuromuscular junctions, a topic that is critical to myasthenia gravis and related diseases, but which has, until now, completely eluded electron microscopic analysis.
- |||||||||| Soliris (eculizumab) / Alexion Pharma
Clinical, Review, Journal: Maintenance immunosuppression in myasthenia gravis, an update. (Pubmed Central) - May 15, 2021
The practical aspects of long-term immunosuppressive therapy in MG are critically reviewed in this article. Application of one or more of these specific therapies is guided based on known efficacy, adverse effect profile, particular disease subtype and severity, and patient co-morbidities.
- |||||||||| Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
Retrospective data, Journal: Differential response to rituximab in anti-AChR and anti-MuSK positive myasthenia gravis patients: a single-center retrospective study. (Pubmed Central) - May 15, 2021
Rituximab treatment response is observed in both AChR+ and MuSK+ patients supporting the role of B cell depletion in the management of MG. While there was no significant difference between these groups in terms of clinical improvement, symptom-free state, and prednisone burden, MuSK+ MG patients may experience greater benefits, including earlier time to remission, fewer exacerbations and hospitalizations post-treatment.
- |||||||||| azathioprine / Generic mfg.
Journal, Adverse events: Myasthenia gravis and azathioprine treatment: Adverse events related to thiopurine S-methyl-transferase (TPMT) polymorphisms. (Pubmed Central) - May 15, 2021
Although TPMT genotype has been associated with AZA-related adverse events, since no statistically significant difference among wild-type and other TPMT genotypes for minor adverse events, our study supports the view that TPMT genotype alone is not enough to adequately personalise the AZA therapy in MG patients. In conclusion, these results were important to characterise the prevalence of TPMT gene variants in MG patients treated with AZA and correlate the adverse events of this therapy in a real-world outpatient clinic from Southern Brazil.
- |||||||||| Review, Journal: ANTIBODIES AND RECEPTORS: from neuromuscular junction to central nervous system. (Pubmed Central) - May 15, 2021
They form the first of a spectrum of diseases in which serum autoantibodies bind to extracellular domains of neuronal proteins throughout the nervous system and lead to constellations of clinical features including paralysis, sensory disturbance and pain, memory loss, seizures, psychiatric disturbance and movement disorders. This review will briefly summarize the ways in which this field has developed, since the 1970s when considerable contributions were made in Ricardo Miledi's laboratory at UCL.
- |||||||||| Clinical, Journal: Exacerbation of myasthenia gravis after amoxicillin therapy: a case series. (Pubmed Central) - May 15, 2021
This review will briefly summarize the ways in which this field has developed, since the 1970s when considerable contributions were made in Ricardo Miledi's laboratory at UCL. We concluded that patients receiving amoxicillin should be closely monitored for possible acute relapse.
- |||||||||| Clinical, Journal: Thymolipoma Association with Myasthenia Gravis: Case Report. (Pubmed Central) - May 15, 2021
Concomitant heart surgery and thymectomy are feasible, and extended thymectomy is the treatment of choice for thymolipoma in myasthenia gravis patients with a better complete remission rate after resection. However, further comparative studies are needed for a more reliable conclusion of the postoperative myasthenia gravis response after resection.
- |||||||||| imatinib / Generic mfg.
Clinical, Review, Journal, Checkpoint inhibition: Neuromuscular complications following targeted therapy in cancer patients: beyond the immune checkpoint inhibitors. Case reports and review of the literature. (Pubmed Central) - May 15, 2021
The first patient was treated with the combination of Vemurafenib and Cobimetinib, BRAF and MEK inhibitors, respectively, for a cutaneous melanoma...The second patient received therapy with Imatinib, tyrosine kinase inhibitor and precursor of the targeted therapy, for a gastrointestinal stromal tumour...DISCUSSION AND We strengthen the relevance of neuromuscular complications which may occur long after treatment start or in patients receiving not only the latest ICPi but also "older" and apparently better-known targeted therapies. Also in the latter cases, an immune-mediated "off-target" pathogenic mechanism can be hypothesized, and consequences can be life threatening, if not promptly diagnosed and appropriately managed.
- |||||||||| sotrastaurin (AEB071) / Novartis
Preclinical, Journal: AEB-071 Ameliorates Muscle Weakness by Altering Helper T Lymphocytes in an Experimental Autoimmune Myasthenia Gravis Rat Model. (Pubmed Central) - May 15, 2021
Although a decrease in the number of Treg cells was observed, the proportion of Th lymphocytes was significantly increased in the EAMG group, and AEB-071 treatment decreased the proportion of Th lymphocytes. CONCLUSIONS We concluded that AEB-071 treatment imparts beneficial effects in EAMG rat models by reducing mortality rate and restoring Th lymphocyte balance, and thus may be an attractive candidate for use in MG treatment.
- |||||||||| mycophenolate mofetil / Generic mfg.
Clinical, Journal: Pyoderma gangrenosum wıth thymoma and myasthenıa gravıs: A case report. (Pubmed Central) - May 15, 2021
The purpose of treatment is; to control the lesions and related diseases for a long time with minimal side effects. Mycophenolate mofetil treatment was used safely and successfully for both generalized MG and PG in our case.
- |||||||||| Clinical, Journal: A rare case of coexistence of autoimmune polyglandular syndrome type 3 with growth hormone deficiency and hyperthyroidism in a patient with pseudo-Turner's syndrome. (Pubmed Central) - May 15, 2021
The principal manifestations of APS-3 are autoimmune thyroid disease and other autoimmune diseases, such as type 1 diabetes, atrophic gastritis, pernicious anemia, vitiligo, alopecia, and myasthenia gravis, but not Addison's disease or hypoparathyroidism. Here we report a case demonstrating the rare coexistence of growth hormone deficiency and hyperthyroidism with sexual dysgenesis, secondary amenorrhea, cardiomegaly, splenomegaly, hypoproteinemia, pleural effusion, seroperitoneum, pericardial effusion, anasarca, osteoporosis, vitamin D deficiency, iron-deficiency anemia, poor blood coagulation, leucocytopenia, peripheral neuropathy, hyperuricemia, ichthyosis, tinea cruris, and onychomycosis.
- |||||||||| dexamethasone injection / Generic mfg.
Journal: Local use of dexamethasone in the treatment of ocular myasthenia gravis. (Pubmed Central) - May 15, 2021
As such, it should be on the differential list for poorly-responsive hypotension following a shift in body positioning under anesthesia. Dexamethasone peribulbar or extraocular muscle injection is effective in the treatment of patients with OMG and may replace systemic drug therapy.
- |||||||||| Review, Journal: Benefit and danger from immunotherapy in myasthenia gravis. (Pubmed Central) - May 15, 2021
This review discusses emerging treatments for MG, including complement inhibitors, neonatal Fc receptor targeting agents, and B cell interfering drugs, focusing on benefit and danger. In the second section of the review, several related adverse events of immunotherapy, including MGonset, are debated.
- |||||||||| Journal: Increasead cholinergic tone causes pre-synaptic neuromuscular degeneration and is associated with impaired diaphragm function. (Pubmed Central) - May 15, 2021
Finally, we showed that ChAT-ChR2-EYFP mice NMJs have compromised safety, possible due to the structural alterations we described. These findings reveal that physiological cholinergic activity is important to maintain the structure and function of the neuromuscular system and help to understand some of the neuromuscular adverse effects experienced by chronically increased NMJ neurotransmission, such as individuals treated with cholinesterase inhibitors.
- |||||||||| Journal: Hemisensory loss in myasthenia gravis. (Pubmed Central) - May 15, 2021
Serum and electrodiagnostic studies confirmed the diagnosis of myasthenia gravis. This case highlights the non-classic presentation of myasthenia gravis in the absence of ophthalmoplegia with a unique unexplained hemisensory deficit.
- |||||||||| Journal: Myasthenia (Pubmed Central) - May 15, 2021
This case highlights the non-classic presentation of myasthenia gravis in the absence of ophthalmoplegia with a unique unexplained hemisensory deficit. No abstract available
- |||||||||| sulfasalazine / Generic mfg.
Review, Journal: Neurology of inflammatory bowel disease. (Pubmed Central) - May 15, 2021
The pathophysiology of neurological manifestations of IBD is mostly immune mediated, but dysfunction of the brain-gut axis, arterial and venous thromboembolism, infections, nutritional deficiencies and side-effects of medications (steroids, metronidazole, sulfasalazine, anti-TNF-α, anti-integrin antibodies) are other contributory mechanisms...Optic neuritis, transverse myelitis, progressive myelopathy, central nervous system infections, epilepsy and encephalopathy are among other uncommon neurological complications. Peripheral nervous system manifestations include peripheral neuropathy, either demyelination and axonal, myasthenia gravis and polymyositis/dermatomyositis and localized forms of myositis.
- |||||||||| Journal: Myasthenia Gravis Revealing Hodgkin's Lymphoma. (Pubmed Central) - May 15, 2021
The association of lymphomas with myasthenia gravis has been rarely reported. Myasthenia gravis is an autoimmune disease of the neuromuscular junction and can be associated with malignant haemopathy.The coexistence of myasthenia and Hodgkin's lymphoma is very rare.This association is a therapeutic challenge.
- |||||||||| Review, Journal: Collagens at the vertebrate neuromuscular junction, from structure to pathologies. (Pubmed Central) - May 14, 2021
A number of neuromuscular diseases, congenital myastenic syndromes and myasthenia gravis are caused by human mutations and autoantibodies against these proteins. Analysis of these diseases and of the specific collagen knock-out mice highlights the roles of some of these collagens in promoting a functional synapse.
- |||||||||| remdesivir / Generic mfg., dexamethasone / Generic mfg.
Clinical, Journal: A case report of COVID-19 in refractory myasthenia: Outcome with remdesivir and dexamethasone. (Pubmed Central) - May 14, 2021
Analysis of these diseases and of the specific collagen knock-out mice highlights the roles of some of these collagens in promoting a functional synapse. This case report illustrates early use of the combination therapy might be beneficial in refractory myasthenia gravis cases even with chronic immunosuppression and severe COVID-19 infection.
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