Symptoma

Juvenile Myasthenia Gravis

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]

Material and Method: This article presents the initial presentation, clinical course, and 2-year follow-up results of 3 patients with juvenile myasthenia gravis, and reviewes the disease in general and current treatment modalities. [ingentaconnect.com]

  • Problems at School

    His parents arrived after his emergency bronchoscopy and provided a detailed history of his problems at school and at home during the preceding 6 months. [anesthesiology.pubs.asahq.org]

  • Foreign Body Aspiration

    Article Information Case Reports Case Reports August 2001 Foreign Body Aspiration: A Presenting Sign of Juvenile Myasthenia Gravis FOREIGN body aspiration is one of the most common pediatric airway emergencies. [anesthesiology.pubs.asahq.org]

  • Infertility

    Thymectomy is not beneficial for MuSK antibody-positive adult or juvenile MG, but azathioprine, mycophenolate mofetil, cyclosporine, and cyclophosphamide, despite risks of infertility and delayed malignancy, are beneficial. [reliasmedia.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]

  • Heart Disease

    An additional 2 subjects died of unrelated causes – 1 of HIV/AIDS and 1 of ischaemic heart disease and arrhythmia, after 3 and 44 years of follow-up, respectively. [samj.org.za]

  • Short Arm

    The strongest genetic determinant is the involvement of genes in the human leukocyte antigen (HLA) complex located on the short arm of chromosome 6 [ 14, 15 ]. The associated HLA alleles vary with the different MG subgroups and with racial origin. [journals.plos.org]

  • Hyperactivity

    Cholinergic neuromuscular hyperactivity in patients with myasthenia gravis seropositive for MuSK antibody. Muscle and Nerve. 2006; 34 (1):111–115. [ PubMed ] [ Google Scholar ] 49. Gronseth GS, Barohn RJ. [ncbi.nlm.nih.gov]

  • Seizure

    Another major disease in addition to myasthenia occurred in 44% of patients; a seizure disorder was the most commonly associated disease. [n.neurology.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]

  • Delayed Bone Age

    Height retardation was related to past cumulative prednisone intake, as was delayed bone age, which was also impacted by age of onset, with delayed bone age more pronounced the earlier the age of onset. [reliasmedia.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]

Advances in the treatment of myasthenia gravis have reduced mortality and morbidity and improved the quality of life in these patients. [squ.pure.elsevier.com]

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]

[…] for the treatment of JMG. [elsevier.es]

Thymectomy has been recommended for the treatment of generalized juvenile MG in patients not responding to anticholinesterase treatment, nor immunomodulatory or immunosuppressive treatments. Age at thymectomy is debated. [orpha.net]

D 26585984 2017 16 Juvenile myasthenia gravis in Norway: A nationwide epidemiological study. 38 Popperud TH...Kerty E 27666466 2017 17 [The pitfalls of using diagnosis and treatment guidelines for patients with juvenile myasthenia gravis]. 38 Ishigaki [malacards.org]

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]

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]

Patients who received thymectomy also likely exhibit a better prognosis [ 6, 12 ]. MuSK myasthenia gravis also had an increased risk of respiratory crises [ 4 ]. [bmcneurol.biomedcentral.com]

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]

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]

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]

Epidemiology of myasthenia gravis: a population-based study in Stockholm, Sweden. Neuroepidemiology. 2002 ;21: 221 - 225. [journals.sagepub.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]

Epidemiology of myasthenia gravis: a population-based study in Stockholm, Sweden. Neuroepidemiology 2002; 21 (5) 221-225 13 Lindner A, Schalke B, Toyka KV. [thieme-connect.com]

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]

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]

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]

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]

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]

Edrophonium prevents antibodies from destroying the neurotransmitter acetylcholine, temporarily relieving muscular weakness. [myastheniagravisnews.com]

Early recognition of this disease helps avoid unnecessary testing, prevent undue parental anxiety, and stop the progression of symptoms. [journals.sagepub.com]

The patient had already been examined at some other centre where he was diagnosed to have congenital ptosis and was advised to undergo tarsofrontalis sling surgery to clear the visual axis and prevent amblyopia. [egms.de]