Juvenile Myasthenia

Anlar, Banu

doi:10.2165/00128072-200002030-00001

Cited by 15 publications

(5 citation statements)

References 37 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Myasthenia crises are unpredictable and responsible for the severity of the disease, affecting respiratory and bulbar muscles. 4 The development of immunomodulatory drugs over the past decades improved prognosis in MG, but these therapies are poorly studied in pediatrics, and the practice is based on adult experiences.…”

mentioning

confidence: 99%

Rituximab Therapy in the Treatment of Juvenile Myasthenia Gravis

et al. 2022

View full text Add to dashboard Cite

Objective:Corticosteroids are the first-line immunosuppressants in the management of juvenile myasthenia gravis despite their adverse effects. The place of new immunosuppressive therapies is not clearly defined by the last international consensus held in March 2019, due to the lack of clinical trials. The aim of this study is to describe the use of rituximab, its efficacy and safety in 8 main pediatric centers of the French neuromuscular reference network in order to propose a new place in the therapeutic strategy of juvenile myasthenia gravis.Method:We conducted a retrospective multicenter study from January 1, 2009 to April 30, 2020, including a large cohort of children with myasthenia gravis in 8 main French pediatric reference centers of the Filnemus network. The type of myasthenia, the different lines of immunosuppressive treatment and the clinical course of the patients were collected. To evaluate the efficacy of rituximab, we studied the clinical course of patients on immunosuppressive therapy. Outcome was defined as the clinical and therapeutic status of patients at the last visit: stable without immunosuppressants, stable with immunosuppressants or unstable.Results:We included 74 patients: 18 children with ocular form and 56 children with generalized form. Of the 37 patients who required immunosuppressive therapy, 27 were treated with rituximab. Patients treated with rituximab had a better outcome than patients treated with conventional immunosuppressants (p = 0.006). The use of rituximab as a first-line immunosuppressant showed a better efficacy with a discontinuation of immunosuppressants in 75% of patients (vs. 25%, p=0.04) and resulted cortisone sparing (42% vs. 92%, p=0.03) compared with rituximab treatment as a second or third-line immunosuppression. Rituximab was well tolerated; no adverse effect was observed.Conclusion:The use of rituximab has increased in France over the last 10 years as first line immunosuppressant. This study suggests a good tolerability and efficacy of rituximab in juvenile myasthenia gravis. Early use appears to improve outcomes and facilitate cortisone-sparing in antibody-positive generalized juvenile myasthenia.Classification of Evidence:This study provides Class III evidence that for children with MG rituximab is effective and well tolerated.

show abstract

mentioning

confidence: 99%

Rituximab Therapy in the Treatment of Juvenile Myasthenia Gravis

et al. 2022

View full text Add to dashboard Cite

show abstract

“…The test is highly specific and becomes increasingly sensitive with increasing age and increasing duration of disease such that about 56% of prepubertal children and up to 82% of peripubertal children with juvenile MG will have detectable antibodies against the acetylcholine receptor [14]. Single fibre EMG is a definitive way of elucidating neuromuscular transmission disorders but is not specific for MG and may well not be tolerated in children [15]. …”

Section: Diagnosismentioning

confidence: 99%

“…If used, they are generally used in relatively large doses carrying the risk of significant side effects—all of which are well documented in children [15]. Other immunomodulatory drugs such as cyclosporine, azathioprine, and cyclophosphamide are rarely used, due to their significant side-effect profile and limited experience in the paediatric setting.…”

Section: Treatmentmentioning

confidence: 99%

“…In the event of an acute deterioration administration of immunoglobulins (IVIGs) or plasma exchange may help to stave off mechanical ventilation [15, 17]. Of the two, plasma exchange is the more effective but also the more laborious to perform, requiring large bore central venous access.…”

Section: Treatmentmentioning

confidence: 99%

“…The thymus gland is implicated in a high proportion of children with juvenile MG. Thymectomy has been shown to bring about improvement in symptoms in between 60–90% of patients undergoing the procedure [9, 15]. The effects of thymectomy may take a period of months or even years to bring about such improvements.…”

Section: Treatmentmentioning

confidence: 99%

See 2 more Smart Citations

Anaesthetic Considerations in Paediatric Myasthenia Gravis

Masters

Bagshaw

2011

Autoimmune Diseases

View full text Add to dashboard Cite

Myasthenia gravis is of particular interest to anaesthetists because of the muscle groups affected, the pharmacology of the neuromuscular junction, and interaction of both the disease and treatment with many anaesthetic drugs. Anaesthetists may encounter children with myasthenia either to facilitate treatment options or to institute mechanical ventilation in the face of a crisis. This paper reviews the literature pertaining to the pathophysiology and applied pharmacology of the disease and explores the relationship between these and the anaesthetic management. In addition to illustrating the tried-and-tested techniques, some newer management options are explored.

show abstract

Treatment outcome in juvenile‐onset myasthenia gravis

et al. 2019

View full text Add to dashboard Cite

Introduction Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction. Approximately 10%–15% of MG patients have juvenile (<18 years of age) onset. We aimed to assess the clinical course, outcome, and subjectively perceived health status of a cohort of juvenile MG patients. Methods This was a retrospective analysis of medical records of 101 patients followed by a cross‐sectional questionnaire study. Results The mean age of patients was 12.8 years at onset and 13.7 years at diagnosis. Ninety percent of the patients were seropositive. Over 40% of the patients were treated with immunosuppression and over 80% underwent thymectomy. The mean Myathenia Gravis Activities of Daily Living (MG‐ADL) scale score was 2.48. At last follow‐up, 30.9% of patients were in complete, stable remission; 77.8% perceived their health as good. Discussion The treatment outcome for juvenile MG is favorable, with a marked reduction of symptoms and good day‐to‐day activity achieved for most patients. Muscle Nerve 59:549–549, 2019

show abstract

Juvenile Myasthenia

Cited by 15 publications

References 37 publications

Rituximab Therapy in the Treatment of Juvenile Myasthenia Gravis

Rituximab Therapy in the Treatment of Juvenile Myasthenia Gravis

Anaesthetic Considerations in Paediatric Myasthenia Gravis

Treatment outcome in juvenile‐onset myasthenia gravis

Contact Info

Product

Resources

About