Myasthenia

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Jianyong Ding - One of the best experts on this subject based on the ideXlab platform.

Lin Wang - One of the best experts on this subject based on the ideXlab platform.

Jason H. Peragallo - One of the best experts on this subject based on the ideXlab platform.

  • Pediatric Myasthenia Gravis
    Seminars in pediatric neurology, 2017
    Co-Authors: Jason H. Peragallo
    Abstract:

    Myasthenia gravis is a disorder of neuromuscular transmission that leads to fatigue of skeletal muscles and fluctuating weakness. Myasthenia that affects children can be classified into the following 3 forms: transient neonatal Myasthenia, congenital myasthenic syndromes, and juvenile Myasthenia gravis (JMG). JMG is an autoimmune disorder that has a tendency to affect the extraocular muscles, but can also affect all skeletal muscles leading to generalized weakness and fatigability. Respiratory muscles may be involved leading to respiratory failure requiring ventilator support. Diagnosis should be suspected clinically, and confirmatory diagnostic testing be performed, including serum acetylcholine receptor antibodies, repetitive nerve stimulation, and electromyography. Treatment for JMG includes acetylcholinesterase inhibitors, immunosuppressive medications, plasma exchange, intravenous immunoglobulins, and thymectomy. Children with Myasthenia gravis require monitoring by a pediatric ophthalmologist for the development of amblyopia from ptosis or strabismus.

Fiona Norwood - One of the best experts on this subject based on the ideXlab platform.

  • Myasthenia in pregnancy best practice guidelines from a uk multispecialty working group
    Journal of Neurology Neurosurgery and Psychiatry, 2014
    Co-Authors: Fiona Norwood, Mandish Dhanjal, Marguerite Hill, N James, Heinz Jungbluth, Pippa Kyle, G Osullivan, J Palace, S Robb, Catherine Williamson
    Abstract:

    A national U.K. workshop to discuss practical clinical management issues related to pregnancy in women with Myasthenia gravis was held in May 2011. The purpose was to develop recommendations to guide general neurologists and obstetricians and facilitate best practice before, during and after pregnancy. The main conclusions were (1) planning should be instituted well in advance of any potential pregnancy to allow time for myasthenic status and drug optimisation; (2) multidisciplinary liaison through the involvement of relevant specialists should occur throughout pregnancy, during delivery and in the neonatal period; (3) provided that their Myasthenia is under good control before pregnancy, the majority of women can be reassured that it will remain stable throughout pregnancy and the postpartum months; (4) spontaneous vaginal delivery should be the aim and actively encouraged; (5) those with severe myasthenic weakness need careful, multidisciplinary management with prompt access to specialist advice and facilities; (6) newborn babies born to myasthenic mothers are at risk of transient myasthenic weakness, even if the mother's Myasthenia is well-controlled, and should have rapid access to neonatal high-dependency support.

  • the use of rituximab in Myasthenia gravis and lambert eaton myasthenic syndrome
    Journal of Neurology Neurosurgery and Psychiatry, 2011
    Co-Authors: Paul Maddison, Fiona Norwood, Heinz Jungbluth, S Robb, John Mcconville, Maria Elena Farrugia, N P Davies, Michael R Rose, David Hiltonjones
    Abstract:

    Aim To assess the treatment effects of rituximab in a population of patients with Myasthenia gravis and Lambert–Eaton myasthenic syndrome. Methods Data on all treated patients in the UK were collected from referring physicians, with full case ascertainment and follow-up. Results Since 2004, 10 patients with generalised Myasthenia gravis (three of whom were positive for muscle-specific tyrosine kinase (MuSK) antibodies) and two patients with Lambert–Eaton myasthenic syndrome (LEMS) were treated with rituximab. Using the Myasthenia Gravis Foundation America postintervention status, three patients (25%) achieved remission, and a further five (42%) improved clinically over an 18-month period. Only one patient developed worsening symptoms. The probability of achieving remission was unrelated to the duration of neurological symptoms prior to treatment. All LEMS and MuSK antibody patients improved following rituximab treatment. Conclusion In a relatively large, unselected group of patients with Myasthenia gravis and LEMS, rituximab treatment resulted in a significant clinical improvement in two-thirds of cases. As a selective, B cell targeted therapy, rituximab should be considered as a treatment option for patients with either Myasthenia gravis or LEMS for whom standard immunosuppressive treatments have been unsuccessful.

Qun Wang - One of the best experts on this subject based on the ideXlab platform.

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