- |||||||||| Journal, Heterogeneity: Heterogeneity and shifts in distribution of muscle weakness in myasthenia gravis. (Pubmed Central) - Aug 27, 2020
MG patients have heterogeneous distributions of muscle weakness and frequently shift between phenotypes. The phenotypic variations found in AChR MG suggest that also other factors aside from the AChR antibody mediated immune response are of importance in determining the disease expression in MG.
- |||||||||| Journal: Update on Ocular Myasthenia Gravis. (Pubmed Central) - Aug 27, 2020
The history and clinical examination are of the utmost importance in correctly identifying OMG patients, as supportive serologic or electrodiagnostic studies are frequently nondiagnostic. In this review, we outline a diagnostic approach to OMG (focusing on key clinical features), discuss therapeutic options, and highlight recent developments in the understanding of OMG.
- |||||||||| Clinical, Journal: A novel case of inclusion body myositis and myasthenia gravis. (Pubmed Central) - Aug 23, 2020
Muscle MRI imaging supported the presence of an inflammatory myopathy and muscle biopsy confirmed the diagnosis of inclusion body myositis. Thus, our patient represents the first reported overlap case of myasthenia gravis and inclusion body myositis.
- |||||||||| Journal: Repetitive ocular vestibular evoked myogenic potentials in myasthenia gravis. (Pubmed Central) - Aug 22, 2020
The RoVEMP test is a new neurophysiologic test that, in contrast to RNS and single-fiber EMG, is able to measure neuromuscular transmission of extraocular muscles, which are the most affected muscles in MG. Especially in diagnostically challenging patients with negative antibody tests, negative RNS results, and isolated ocular muscle weakness, the RoVEMP test has a clear added value in supporting the diagnosis of MG.
- |||||||||| tacrolimus / Generic mfg., Soliris (eculizumab) / Alexion Pharma, cyclophosphamide intravenous / Generic mfg.
Retrospective data, Journal: Immunosuppressive and monoclonal antibody treatment for myasthenia gravis: A network meta-analysis. (Pubmed Central) - Aug 19, 2020
These findings demonstrated that ECZ represented the most effective and tolerable therapeutic alternative to be recommended for refractory MG. TAC may be a beneficial therapy to treat MG extensively while the efficacy of CsA and cyclophosphamide may be limited by their multiple or severe AEs.
- |||||||||| Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
Review, Journal: Rituximab in AChR subtype of myasthenia gravis: systematic review. (Pubmed Central) - Aug 18, 2020
P2
Future studies should take into account the subtype of MG and employ reliable measures of outcome and severity focusing on how to identify patients who may benefit from the treatment. Trial registration number: NCT02110706.
- |||||||||| Journal: Therapeutic Plasma Exchange as a Treatment for Autoimmune Neurological Disease. (Pubmed Central) - Aug 18, 2020
After TPE, 99 patients (52.9%) showed improvement in the mRS scores and a statistical significance (p < 0.05) was seen between the admission score and after TPE for every diagnosis except for CIDP. TPE has an adequate safety profile, and improvement in functionality in treated patients reflects its effectiveness.
- |||||||||| Review, Journal: A Review of Psychiatric Comorbidity in Myasthenia Gravis. (Pubmed Central) - Aug 18, 2020
Although treatments for the somatic effects of myasthenia gravis have evolved over the past century, the paradigm of clinical practice has yet to adequately address the management of psychological impacts on the disease. This review is hoped to raise the necessary awareness in this regard.
- |||||||||| Spinraza (nusinersen) / Biogen, Ionis, Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
Clinical, Review, Journal: Management of patients with neuromuscular disorders at the time of the SARS-CoV-2 pandemic. (Pubmed Central) - Aug 18, 2020
In addition, specific drugs for SARS-CoV2 infection under investigation impair neuromuscular function significantly; chloroquine and azithromycin are not recommended in myasthenia gravis without available ventilatory support and prolonged prone positioning may influence options for treatment...Thus, as the first wave of the pandemic is progressing, clinicians and researchers should address these crucial open issues to ensure adequate caring for NMD patients. This manuscript summarizes available evidence so far and provides guidance for both general neurologists and NMD specialists dealing with NMD patients in the time of COVID-19.
- |||||||||| Soliris (eculizumab) / Alexion Pharma, Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
[VIRTUAL] Safety of eculizumab in NMOSD and MG – analysis of the phase 3 studies PREVENT and REGAIN and their extensions () - Aug 16, 2020 - Abstract #MSDC2020MSDC_812;
P3
One patient with gMG (2 ISTs) had meningococcal meningitis that resolved with antibiotics and eculizumab was resumed.Conclusions In AQP4+ NMOSD and AChR+ gMG, infection rates/100 PY were similar in eculizumab and placebo groups, regardless of concomitant IST. Infection rates/100 PY were consistent with the established safety profile of eculizumab.
- |||||||||| Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
Clinical, Review, Journal: MuSK-Associated Myasthenia Gravis: Clinical Features and Management. (Pubmed Central) - Aug 15, 2020
However, the majority of MuSK-MG patients are refractory to treatment. In these cases, the use of rituximab showed promising results, resulting in sustained symptom control.
- |||||||||| Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
Review, Journal: Management of Juvenile Myasthenia Gravis. (Pubmed Central) - Aug 15, 2020
Second-line therapies including azathioprine and mycophenolate may be considered where there is: no response to steroids, inability to wean to a reasonable minimum effective dose or if side-effects are intolerable...Muscle-specific tyrosine kinase (MuSK)-JMG show a poorer response to pyridostigmine and anecdotal evidence suggests that rituximab should be considered as second-line immunosuppression...Key questions remain in the management of JMG: when to initiate both first- and second-line treatments, choosing between steroid-sparing agents, and determining the optimal dose and treatment duration. We feel that given the rarity of this disease, the establishment of national registries and collaboration across groups will be needed to address these issues and facilitate future drug trials in JMG.
- |||||||||| Review, Journal: Class IX Myosins: Motorized RhoGAP Signaling Molecules. (Pubmed Central) - Aug 13, 2020
These cellular functions affect embryonic development, adult organ homeostasis and immune responses. Human diseases associated with mutations in the two class IX myosins, Myo9a and Myo9b, have been identified, including hydrocephalus and congenital myasthenic syndrome in connection with Myo9a and autoimmune diseases in connection with Myo9b.
- |||||||||| tofacitinib / Generic mfg.
Enrollment open: Safety And Efficacy Of Tofacitinib In Patients With Refractory Myasthenia Gravis: A Pilot Study (clinicaltrials.gov) - Aug 12, 2020
P1, N=5, Recruiting,
Human diseases associated with mutations in the two class IX myosins, Myo9a and Myo9b, have been identified, including hydrocephalus and congenital myasthenic syndrome in connection with Myo9a and autoimmune diseases in connection with Myo9b. Not yet recruiting --> Recruiting
- |||||||||| tropicamide / Generic mfg.
Journal: Exacerbation of ocular myasthenia gravis (Pubmed Central) - Aug 11, 2020
This case report describes an exacerbation of ocular myasthenia gravis after intravitreal injection using local anesthesia. A possible association with local therapeutic agents is critically discussed but cannot be excluded.
- |||||||||| Clinical, Journal: Case report: cardiac herniation following robotic-assisted thymectomy. (Pubmed Central) - Aug 11, 2020
This potentially fatal complication, although rare, should always be considered whenever there is hemodynamic instability entry or resection of the pericardium during surgery. We now routinely sew in a pericardial patch using our robotic surgical system for any defect over 3 cm × 3 cm that extends from the mid- to inferior portions of the heart.
- |||||||||| Trial completion date, Trial primary completion date: 3,4-Diaminopyridine Use in Lambert-Eaton Myasthenic Syndrome(LEMS) and Congenital Myasthenic Syndromes (CMS) (clinicaltrials.gov) - Aug 11, 2020
P=N/A, N=22, Active, not recruiting,
Taken together, the distinct pattern of disease association and the unique amino acid sequence of the third hypervariable region of the HLA-DRB1 provide some hints on how HLA-DRB1*16:02 is involved in the pathogenesis of autoimmune diseases. Trial completion date: Jun 2020 --> Jun 2021 | Trial primary completion date: Jun 2020 --> Jun 2021
- |||||||||| Review, Journal, Checkpoint inhibition: Immune Checkpoint Inhibitor-Induced Myasthenia Gravis. (Pubmed Central) - Aug 9, 2020
Treatment plans should be individualized on the basis of the extent of organ involvement and clinical severity. Additional therapeutic studies on irMG in the future are required to minimize irAE-related mortality and increase the safety of patients with cancer in the ICI era.
- |||||||||| Journal: New Insights into the Exploitation of Vitis vinifera L. cv. Aglianico Leaf Extracts for Nutraceutical Purposes. (Pubmed Central) - Aug 8, 2020
Regarding enzymatic inhibitory activity, ASE 50 (IC = 107.16 ± 8.12 μg/mL) and SOX (IC = 171.34 ± 12.12 μg/mL) extracts exhibited the highest AChE and BChE inhibitory activity, respectively, while UAE (IC = 293.2 ± 25.6 μg/mL, followed by SOX (IC = 302.5 ± 38.3 μg/mL) showed the highest tyrosinase inhibition value. Our results demonstrated for the first time that Aglianico leaves are important sources of phenols that could be used to prevent oxidative stress and be potentially helpful in diseases treatable with tyrosinase and cholinesterase inhibitors, like myasthenia gravis or Alzheimer's.
- |||||||||| Clinical, Journal: Takotsubo Cardiomyopathy associated with myasthenic crisis: An Illustrative Case. (Pubmed Central) - Aug 8, 2020
Our results demonstrated for the first time that Aglianico leaves are important sources of phenols that could be used to prevent oxidative stress and be potentially helpful in diseases treatable with tyrosinase and cholinesterase inhibitors, like myasthenia gravis or Alzheimer's. No abstract available
|
