Paediatric presentation of MG is more common in Oriental than in Caucasian populations [5]. Up to 50% of all cases of MG in Chinese populations present in childhood, mostly with ocular features, with a peak age at presentation of 5–10 years [6]. [hindawi.com]

We present four consecutive cases of JMG presenting with predominant bulbar symptoms including voice nasality, dysarthria, and swallowing difficulty (even leading to significant weight loss). [dergipark.org.tr]

Presenting features Presenting features of the 5 patients attributable to JSLE, n (%) Joint involvement 5/5 (100) Fatigue/weight loss/fever 4/5 (80) Proliferative GN 3/5 (60) Skin involvement 2/5 (40) Cytopenias 2/5 (40) Presenting features of the 5 patients [academic.oup.com]

Up to 50% of all cases of myasthenia in Chinese population present in childhood [8], while in Caucasians only 10% of the cases present with prepubertal onset [9]. [egms.de]

Design/Methods: Retrospective chart review of electronic medical records of all children with ICD9/10 diagnosis of myasthenia gravis presenting to Texas Children’s Hospital. Results: The average age at presentation in this cohort was 8.2 years. [n.neurology.org]

• Pleural Effusion

  The only postoperative complication was pleural effusion in one patient; fluid was removed by pleural puncture. During the postoperative period, the dose of parasympathomimetics was reduced and the associated adverse effects were monitored. [elsevier.es]

• Liver Dysfunction

  The side-effects seen with azathioprine use include GI disturbance, liver dysfunction and myelosuppression. [frontiersin.org]

• Thrombosis

  Side-effects include infusion reactions, rash, headache, hypertension, increased risk of thrombosis and aseptic meningitis. [frontiersin.org]

• Withdrawn

  At this point she was 12 months into her diagnosis, while higher-dose steroids induced remission she was developing intolerable side-effects and was becoming depressed and withdrawn. [frontiersin.org]

• Meningism

  Side-effects include infusion reactions, rash, headache, hypertension, increased risk of thrombosis and aseptic meningitis. [frontiersin.org]

• Myokymia

  The presence of both myokymia and fasciculations can also be indicative of this condition. Similar to adult MuSK-MG there is a strong female predominance with 89% of cases female in the largest published pediatric case series (20). [frontiersin.org]

Keywords juvenile myasthenia gravis - clinical symptoms - differential diagnosis - diagnostic workup - therapy References 1 Erb W. Zur Kasuistik der bulbaeren Lähmungen. Ueber einen neuen, wahrscheinlich bulbaeren Symptomenkomplex. [thieme-connect.com]

• Pleural Effusion

  The only postoperative complication was pleural effusion in one patient; fluid was removed by pleural puncture. During the postoperative period, the dose of parasympathomimetics was reduced and the associated adverse effects were monitored. [elsevier.es]

Although most of the current practice on diagnosis and treatment of JMG in children is extrapolated from adult trials and experience, there are important differences related to diagnosis and treatment of children with JMG Few paediatric neurologists treat [enmc.org]

Treatment for JMG is symptomatic and aetiological. [elsevier.es]

Given the evidence that prepubertal JMG may behave quite differently in terms of disease severity and progression, this may impact on necessity for treatment and treatment response. [hindawi.com]

It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis or treatment. [myastheniagravisnews.com]

The only treatment-related side-effect was transient paresthesia, which were reported in 60%, but did not lead to discontinuation of treatment. [frontiersin.org]

In general, the long-term prognosis of younger patients with juvenile MG is good. Children with the condition — especially those who develop the disease before puberty — have higher rates of disease remission compared with adults. [myastheniagravisnews.com]

Prognosis Severe cases may be life-threatening, but the prognosis is generally very good. In the prepuberal form, there is a higher rate of remission in patients with the ocular form. [orpha.net]

Rowland, “Prognosis of ocular myasthenia,” Annals of Neurology, vol. 14, no. 5, pp. 516–519, 1983. View at: Google Scholar S. L. Pineles, R. A. Avery, H. E. [hindawi.com]

Prognosis of ocular myasthenia. Ann Neurol 1983;14:516-9. 11. Pineles SL, Avery RA, Moss HE, Finkel R, Blinman T, Kaiser L, et al. Visual and systemic outcomes in pediatric ocular myasthenia gravis. Am J Ophthalmol 2010;150:453-9. [smjonline.org]

Prognosis of ocular myasthenia. Annals of Neurology. 1983; 14 (5):516–519. [ PubMed ] [ Google Scholar ] 16. Pineles SL, Avery RA, Moss HE, et al. Visual and systemic outcomes in pediatric ocular myasthenia gravis. [ncbi.nlm.nih.gov]

Etiology The exact pathogenesis is not known but MG is related to circulating antibodies to various muscle receptors, including, in most of patients, acetylcholine receptor (AChR) and, rarely, muscle-specific receptor tyrosine kinase (MuSK). [orpha.net]

Myasthenia gravis: A comprehensive review of immune dysregulation and etiological mechanisms. J Autoimmun. 2014;52:90–100. pmid:24389034 View Article PubMed/NCBI Google Scholar 7. Namba T, Brunner NG, Brown SB, Muguruma M, Grob D. [journals.plos.org]

The most common etiology among patients with juvenile myasthenia gravis is the production of antibodies against the acetylcholine receptor. [bmcneurol.biomedcentral.com]

Juvenile myasthenia gravis shares a similar pathophysiologic origin with adult myasthenia gravis, but there are important differences, mostly relating to epidemiology, presentation, and therapeutic decision making. [pptaglobal.org]

USA Search for more papers by this author First published: 18 March 2009 Cited by: 60 Abstract Juvenile myasthenia gravis shares a similar pathophysiologic origin with adult myasthenia gravis, but there are important differences, mostly relating to epidemiology [onlinelibrary.wiley.com]

The epidemiology of myasthenia gravis. Ann N Y Acad Sci. 2003;998:407–412. 2. Heldal AT, Owe JF, Gilhus NE, et al. Seropositive myasthenia gravis: A nationwide epidemiologic study. Neurology. 2009;73(2):150–151. 3. Phillips LH. [jems.com]

Epidemiology and Clinical Features JMG is a rare disorder of childhood, but its incidence and prevalence vary geographically. Precise data on incidence and prevalence are not known. [hindawi.com]

Summary Epidemiology The exact prevalence and incidence of juvenile MG are not known. Estimated incidence has been reported at 1/1,000,000 to 1/200,000. [orpha.net]

Juvenile myasthenia gravis shares a similar pathophysiologic origin with adult myasthenia gravis, but there are important differences, mostly relating to epidemiology, presentation, and therapeutic decision making. [pptaglobal.org]

Neurology, Children's Hospital Boston, Harvard Medical School, 300 Longwood Avenue, Boston, Massachusetts 02115, USA Search for more papers by this author First published: 18 March 2009 Cited by: 60 Abstract Juvenile myasthenia gravis shares a similar pathophysiologic [onlinelibrary.wiley.com]

Neonatal myasthenia caused by maternal MG Maternal MG can cause transient neonatal myasthenia in about 20% of cases, the pathophysiology behind is AChR autoantibodies cross the placenta from the mother to the fetus. [intechopen.com]

To some extent, treatment options for adults may be adapted for children, but specific pathophysiology differences must be considered in this population group. [lupinepublishers.com]

Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test Myasthenia gravis (MG) is an acquired disorder of neuromuscular transmission caused by the binding of pathogenic autoantibodies [mayocliniclabs.com]

Early diagnosis is imperative for delaying the progression of the disease, and prevention and adequately management the myasthenic crisis. [ingentaconnect.com]

In JMG, abnormal antibodies attach to the muscle, and prevent acetylcholine from working correctly. Eventually, the lack of nerve transmission leads to muscle weakness. These abnormal antibodies are made by the immune system. [chop.edu]

Treatments for myasthenia gravis include medications that: Prevent the body from making too many abnormal antibodies, or Improve connections between the nerves and muscles Other treatments may filter out abnormal antibodies or rely on donated blood to [childrens.com]