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Juvenile Myasthenia Gravis


Presentation

  • 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]
  • Presentation was ocular in 63% of patients. Another major disease in addition to myasthenia occurred in 44% of patients; a seizure disorder was the most commonly associated disease.[n.neurology.org]
  • This child’s clinical presentation and response to treatment indicate some of the differences in presenting symptoms and signs and response to thymectomy in JMG compared with adult-onset myasthenia gravis.[anesthesiology.pubs.asahq.org]
  • Ethnicity has influence on incidence, clinical presentation, and the course of the disease.[semanticscholar.org]
  • One case presented with dysphagia, while another case presented with dysarthria. Two patients presented with respiratory muscle weakness at onset, and 14 (4.3%) presented weakness in two or more muscle groups at onset (Table 2 ). Table 2.[frontiersin.org]
Foreign Body Aspiration
  • body aspiration: a presenting sign of juvenile myasthenia gravis. ( 11506135 ) Murray DJ...McAllister J 2001 47 Video-assisted thoracoscopic thymectomy in juvenile myasthenia gravis. ( 11510927 ) Kolski HK...Vajsar J 2001 48 High-dose intravenous immunoglobulin[malacards.org]
Anemia
  • These tumors are associated with autoimmune diseases in up to 50% of the patients, including myasthenia gravis, aplastic anemia, hypogammaglobulinemia, lichen planus, and Graves' disease. [2] Less than 200 cases have been described in the literature.[smjonline.org]
Aspiration
  • We report an 8-yr-old boy who aspirated popcorn during a myasthenic crisis. This foreign body aspiration led to the subsequent diagnosis and treatment for juvenile myasthenia gravis (JMG).[anesthesiology.pubs.asahq.org]
  • […] gravis. ( 12776235 ) Anlar B...Vincent A 2003 44 Thoracoscopic thymectomy for juvenile myasthenia gravis. ( 12522275 ) Kumar A...Kalara V 2002 45 [Thymectomy in juvenile myasthenia gravis]. ( 12221621 ) Camacho-Salas A...Medina M 2002 46 Foreign body aspiration[malacards.org]
  • Children are at risk of choking or aspiration and are at increased risk of chest infection. Occasionally, impairment of the respiratory muscles necessitates ventilatory support. This is known as “myasthenic crisis”.[lupinepublishers.com]
Hypoventilation
  • […] deterioration en route to the operating room was initially believed to be the result of complete airway obstruction from movement of the foreign body, but his rapid response to bag and mask ventilation suggested that an underlying neurologic condition caused hypoventilation[anesthesiology.pubs.asahq.org]
Tachypnea
  • At the time of arrival in the emergency room, clinical examination indicated stridor, tachypnea, and poor air entry by auscultation. His oxygen saturation was 100% with 5 l O 2 delivered via a nonrebreathing mask.[anesthesiology.pubs.asahq.org]
Nystagmus
  • Open-loop and closed-loop optokinetic nystagmus (OKN) in myasthenia gravis and nonmyasthenic subjects. Exp Neurol. 2000 Nov. 166(1):166-72. [Medline]. Movaghar M, Slavin ML.[medscape.com]

Workup

  • 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]
Mediastinal Mass
  • Asymptomatic giant mediastinal mass: A rare case of thymolipoma. J Bras Pneumol 2009;35:1806-8. 2. Rieker RJ, Schirmacher P, Schnabel PA, Moser K, Hoffmann H,Dienemann H, et al. Thymolipoma.[smjonline.org]

Treatment

  • 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]
  • 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]
  • […] n D 2017 7 Juvenile myasthenia gravis in Norway: A nationwide epidemiological study. ( 27666466 ) Popperud TH...Kerty E 2017 8 The role of thymectomy in the treatment of juvenile myasthenia gravis: a systematic review. ( 28401300 ) Madenci AL...Weldon[malacards.org]
  • JMG can also spontaneously go into remission (no symptoms are shown and no treatment is necessary) or improve. Treatment options depend on a person’s symptoms, and include: Acetylcholenesterase inhibitors: These medicines (ex.[youngwomenshealth.org]
  • 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]

Prognosis

  • 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]
  • 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

  • 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]

Epidemiology

  • 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]
  • 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]
  • […] n D 2017 7 Juvenile myasthenia gravis in Norway: A nationwide epidemiological study. ( 27666466 ) Popperud TH...Kerty E 2017 8 The role of thymectomy in the treatment of juvenile myasthenia gravis: a systematic review. ( 28401300 ) Madenci AL...Weldon[malacards.org]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • 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]
  • A major understanding of the underlying pathophysiology of autoimmune diseases has been the application of genome wide association scans that have identified a degree of genetic sharing among the autoimmune diseases. [10] Autoimmunity, on the other hand[en.wikipedia.org]
  • Pathophysiology Depolarization of a motor neuron propagates to the axon terminal, where acetylcholine is released into the synapse.[aao.org]

Prevention

  • 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]
  • 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]

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