- |||||||||| Clinical, Journal: Targeted Treatments for Inherited Neuromuscular Diseases of Childhood. (Pubmed Central) - Jul 7, 2021
This review highlights these advances and emerging targeted therapies for Duchenne muscular dystrophy and centronuclear myopathy, while also covering enzyme replacement therapy and small molecule-based targeted therapies for conditions such as Pompe's disease and congenital myasthenic syndromes. With these and other newer techniques for targeted correction of genetic defects, such as CRISPR/Cas9, there is now hope that treatments for many more genetic diseases of the nervous system will follow in the near future.
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Clinical, Journal, IO biomarker: Clinical Profile and Treatment Response in Patients with CASPR2 Antibody-Associated Neurological Disease. (Pubmed Central) - Jul 6, 2021 Patients were treated with intravenous methylprednisolone and plasma exchange during the acute symptomatic phase followed by pulsed intravenous methyl prednisolone to maintain remission...CASPR2 antibody-associated disease has favorable response to immunotherapy with complete improvement and good outcome. Underlying malignancy may be a marker for poor prognosis.
- |||||||||| Journal: Effects of acute neurologic disease on the heart. (Pubmed Central) - Jul 2, 2021
Although typically reversible, these cardiac complications are important to recognize as they are associated with increased morbidity and mortality. In this chapter, we discuss the suspected pathophysiology, clinical presentation, and management of the cardiac dysfunction that occur as a consequence of different types of acute neurologic illness.
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Review, Journal: History of Myasthenia Gravis Revisited. (Pubmed Central) - Jul 1, 2021 Treatment modalities which completely changed the prognosis of MG, including positive pressure mechanic ventilation and corticosteroids as well as plasma exchange/IVIg and azathioprine, were put to use...Valuable guidelines were published. In the last part of the review, the experience in MG of Istanbul University, a pioneer in Turkey, is related.
- |||||||||| Journal: Neuromuscular disorders in pregnancy. (Pubmed Central) - Jul 1, 2021
Many commonly used therapies have fetal animal but no proven human toxicity concerns, complicating treatment and risk decisions. Weaning off effective therapeutic agents or preemptive aggressive treatment or surgery prior to planned pregnancy is an option in some conditions.
- |||||||||| Clinical, Observational data, Journal: Diagnostic yields and clinical features of ocular myasthenia gravis. (Pubmed Central) - Jul 1, 2021
Unequivocal response to pyridostigmine was observed in 31 (86.11%) patients. For seropositive cases, AchR Ab titer was significantly higher in the group with 2 clinical symptoms than that in the 1 clinical symptom (P = .011).This study presents the usefulness and diagnostic validity of antimyasthenic treatment for OMG, especially seronegative OMG, with detailed symptom analysis.
- |||||||||| Review, Journal: Fc-Receptor Targeted Therapies for the Treatment of Myasthenia gravis. (Pubmed Central) - Jun 30, 2021
Furthermore, we evaluate prospects of therapies targeting classical Fc receptors that have shown promising therapeutic efficacy in other antibody-mediated conditions. Increased availability of Fc- and Fc receptor-targeting biologics might foster the development of personalized immunotherapies with the potential to induce sustained disease remission in patients with MG.
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Clinical, Review, Journal: Neurocritical Care of the Pregnant Patient. (Pubmed Central) - Jun 29, 2021 Early studies on hypothermia, ketamine, and immunomodulating therapies in pregnancy are promising...When difficult decisions regarding preterm delivery arise, recent outcome studies can help inform discussion. Lastly, when the feared complication of maternal death by neurologic criteria occurs, fetal somatic support may help to save at least one life.
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Review, Journal: Inebilizumab: First Approval. (Pubmed Central) - Jun 26, 2021 The drug is also undergoing clinical evaluation for kidney transplant desensitization, myasthenia gravis, and IgG4-related disease. This article summarizes the milestones in the development of inebilizumab leading to this first approval for the treatment of AQP4-IgG seropositive NMOSD.
- |||||||||| Clinical, Journal: Epidemiology of Myasthenia Gravis in Sweden 2006-2016. (Pubmed Central) - Jun 26, 2021
This article summarizes the milestones in the development of inebilizumab leading to this first approval for the treatment of AQP4-IgG seropositive NMOSD. The incidence and prevalence of Myasthenia Gravis have increased over time in Sweden, and the rates are high in comparison with other countries.
- |||||||||| Clinical, Journal: Myasthenia gravis posterior to thymectomy: Case report. (Pubmed Central) - Jun 26, 2021
The late onset of MG and other autoimmune disorders should be considered as possible complications of the surgical treatment of thymomas. Therefore, after the resection of a thymoma, follow-up care with post-operative MG consideration is necessary.
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Journal, Checkpoint inhibition: Immune Checkpoint Inhibitor-Mediated Myasthenia Gravis. (Pubmed Central) - Jun 24, 2021 He was immediately commenced on 1 mg/kg of intravenous methylprednisolone and pyridostigmine 60 mg 3 times a day...WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Immune-mediated myasthenia gravis is an important toxicity of ICIs. Early recognition and treatment of this presentation may reduce the significant morbidity and mortality associated with it.
- |||||||||| Review, Journal: Classification of myasthenia gravis and congenital myasthenic syndromes in dogs and cats. (Pubmed Central) - Jun 24, 2021
Congenital myasthenic syndromes are classified according to the affected NMJ component, the mechanism of the defect of neuromuscular transmission, the affected protein, and ultimately the mutated gene responsible. In proposing this categorization of MG and CMSs, we hope to aid recognition of the disease groups for both conditions, as well as guide treatment, refine prognosis, and provide a framework for additional studies of these conditions.
- |||||||||| Journal: Imbalance of Th17 and Tregs in thymoma may be a pathological mechanism of myasthenia gravis. (Pubmed Central) - Jun 23, 2021
Overall, Th17 cells and related transcription factors and cytokines in thymoma with MG patients were higher than those in thymoma patients, whereas, Tregs showed the opposite results, the mechanism may be that thymoma can secrete IL6 and IL21. These findings indicated that imbalances in Th17/Tregs may account for the pathogeny of thymoma with MG.
- |||||||||| Clinical, Journal: Acute Neuromuscular Disorders in the Pediatric Intensive Care Unit. (Pubmed Central) - Jun 22, 2021
Although acute and acute-on-chronic neuromuscular disorders are encountered relatively rarely in the PICU, familiarity with the presenting features of these disorders is important in facilitating timely diagnosis. This, in turn, enables the institution of effective management strategies, thereby avoiding complications associated with diagnostic delays.
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