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Juvenile myasthenia gravis

MedGen UID:
1842990
•Concept ID:
C5680023
•
Disease or Syndrome
Synonyms: childhood myasthenia gravis; Childhood myasthenia gravis; Juvenile acquired myasthenia; juvenile acquired myasthenia; Juvenile autoimmune myasthenia gravis; juvenile autoimmune myasthenia gravis; juvenile myasthenia gravis; myasthenia gravis of childhood; paediatric myasthenia gravis; pediatric myasthenia gravis
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018325
Orphanet: ORPHA391497

Definition

Juvenile myasthenia gravis (MG; see this term) is a rare form of MG, an autoimmune disorder of the neuromuscular junction resulting in ocular manifestations or generalized weakness, with onset before 18 years of age. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVJuvenile myasthenia gravis

Professional guidelines

PubMed

Treatment of juvenile myasthenia gravis with tacrolimus: A cohort study.
Wang G, Kessi M, Huang X, Zhang W, Zhang C, He F, Peng J, Yin F, Yang L
Eur J Neurol 2024 Dec;31(12):e16466. Epub 2024 Sep 4 doi: 10.1111/ene.16466. PMID: 39230556Free PMC Article
Rituximab Therapy in the Treatment of Juvenile Myasthenia Gravis: The French Experience.
Molimard A, Gitiaux C, Barnerias C, Audic F, Isapof A, Walther-Louvier U, Cances C, Espil-Taris C, Davion JB, Quijano-Roy S, Grisel C, Chabrol B, Desguerre I
Neurology 2022 Jun 7;98(23):e2368-e2376. Epub 2022 Mar 21 doi: 10.1212/WNL.0000000000200288. PMID: 35314497
Juvenile myasthenia gravis: recommendations for diagnostic approaches and treatment.
Della Marina A, Trippe H, Lutz S, Schara U
Neuropediatrics 2014 Apr;45(2):75-83. Epub 2014 Jan 27 doi: 10.1055/s-0033-1364181. PMID: 24470240

Recent clinical studies

Etiology

Rituximab in juvenile myasthenia gravis-an international cohort study and literature review.
Ramdas S, Della Marina A, Ryan MM, McWilliam K, Klein A, Jacquier D, Alabaf S, Childs AM, Parasuraman D, Beeson D, Palace J, Jungbluth H
Eur J Paediatr Neurol 2022 Sep;40:5-10. Epub 2022 Jun 25 doi: 10.1016/j.ejpn.2022.06.009. PMID: 35835035
Outcome after Robotic-Assisted Thymectomy in Children and Adolescents with Acetylcholine Receptor Antibody-Positive Juvenile Myasthenia Gravis.
Della Marina A, Kölbel H, Müllers M, Kaiser O, Ismail M, Swierzy M, Rueckert JC, Schara U
Neuropediatrics 2017 Aug;48(4):315-322. Epub 2017 Jun 19 doi: 10.1055/s-0037-1603775. PMID: 28628938
Juvenile myasthenia gravis.
Chiang LM, Darras BT, Kang PB
Muscle Nerve 2009 Apr;39(4):423-31. doi: 10.1002/mus.21195. PMID: 19229875
Juvenile myasthenia gravis.
Snead OC 3rd, Benton JW, Dwyer D, Morley BJ, Kemp GE, Bradley RJ, Oh SJ
Neurology 1980 Jul;30(7 Pt 1):732-9. doi: 10.1212/wnl.30.7.732. PMID: 7190239
Thymectomy in juvenile myasthenia gravis.
Seybold ME, Howard FM Jr, Duane DD, Payne WS, Harrison EG Jr
Arch Neurol 1971 Nov;25(5):385-92. doi: 10.1001/archneur.1971.00490050019001. PMID: 5110884

Diagnosis

Pediatric Neuromuscular Diseases.
Rathore G, Kang PB
Pediatr Neurol 2023 Dec;149:1-14. Epub 2023 Aug 29 doi: 10.1016/j.pediatrneurol.2023.08.034. PMID: 37757659
Pediatric Myasthenia Gravis.
Peragallo JH
Semin Pediatr Neurol 2017 May;24(2):116-121. Epub 2017 Apr 7 doi: 10.1016/j.spen.2017.04.003. PMID: 28941526
Juvenile myasthenia gravis.
Chiang LM, Darras BT, Kang PB
Muscle Nerve 2009 Apr;39(4):423-31. doi: 10.1002/mus.21195. PMID: 19229875
Juvenile myasthenia gravis: an unusual presentation.
Winter PH, Koopmann CF Jr
Int J Pediatr Otorhinolaryngol 1990 Aug;19(3):273-6. doi: 10.1016/0165-5876(90)90007-e. PMID: 2210954
Juvenile myasthenia gravis.
Snead OC 3rd, Benton JW, Dwyer D, Morley BJ, Kemp GE, Bradley RJ, Oh SJ
Neurology 1980 Jul;30(7 Pt 1):732-9. doi: 10.1212/wnl.30.7.732. PMID: 7190239

Therapy

Rituximab in juvenile myasthenia gravis-an international cohort study and literature review.
Ramdas S, Della Marina A, Ryan MM, McWilliam K, Klein A, Jacquier D, Alabaf S, Childs AM, Parasuraman D, Beeson D, Palace J, Jungbluth H
Eur J Paediatr Neurol 2022 Sep;40:5-10. Epub 2022 Jun 25 doi: 10.1016/j.ejpn.2022.06.009. PMID: 35835035
Rituximab Therapy in the Treatment of Juvenile Myasthenia Gravis: The French Experience.
Molimard A, Gitiaux C, Barnerias C, Audic F, Isapof A, Walther-Louvier U, Cances C, Espil-Taris C, Davion JB, Quijano-Roy S, Grisel C, Chabrol B, Desguerre I
Neurology 2022 Jun 7;98(23):e2368-e2376. Epub 2022 Mar 21 doi: 10.1212/WNL.0000000000200288. PMID: 35314497
Outcome after Robotic-Assisted Thymectomy in Children and Adolescents with Acetylcholine Receptor Antibody-Positive Juvenile Myasthenia Gravis.
Della Marina A, Kölbel H, Müllers M, Kaiser O, Ismail M, Swierzy M, Rueckert JC, Schara U
Neuropediatrics 2017 Aug;48(4):315-322. Epub 2017 Jun 19 doi: 10.1055/s-0037-1603775. PMID: 28628938
Juvenile myasthenia gravis.
Chiang LM, Darras BT, Kang PB
Muscle Nerve 2009 Apr;39(4):423-31. doi: 10.1002/mus.21195. PMID: 19229875
Juvenile myasthenia gravis.
Snead OC 3rd, Benton JW, Dwyer D, Morley BJ, Kemp GE, Bradley RJ, Oh SJ
Neurology 1980 Jul;30(7 Pt 1):732-9. doi: 10.1212/wnl.30.7.732. PMID: 7190239

Prognosis

Rituximab Therapy in the Treatment of Juvenile Myasthenia Gravis: The French Experience.
Molimard A, Gitiaux C, Barnerias C, Audic F, Isapof A, Walther-Louvier U, Cances C, Espil-Taris C, Davion JB, Quijano-Roy S, Grisel C, Chabrol B, Desguerre I
Neurology 2022 Jun 7;98(23):e2368-e2376. Epub 2022 Mar 21 doi: 10.1212/WNL.0000000000200288. PMID: 35314497
Outcome after Robotic-Assisted Thymectomy in Children and Adolescents with Acetylcholine Receptor Antibody-Positive Juvenile Myasthenia Gravis.
Della Marina A, Kölbel H, Müllers M, Kaiser O, Ismail M, Swierzy M, Rueckert JC, Schara U
Neuropediatrics 2017 Aug;48(4):315-322. Epub 2017 Jun 19 doi: 10.1055/s-0037-1603775. PMID: 28628938
Juvenile myasthenia gravis: recommendations for diagnostic approaches and treatment.
Della Marina A, Trippe H, Lutz S, Schara U
Neuropediatrics 2014 Apr;45(2):75-83. Epub 2014 Jan 27 doi: 10.1055/s-0033-1364181. PMID: 24470240
Outcome of children with acetylcholine receptor (AChR) antibody positive juvenile myasthenia gravis following thymectomy.
Heng HS, Lim M, Absoud M, Austin C, Clarke D, Wraige E, Reid C, Robb SA, Jungbluth H
Neuromuscul Disord 2014 Jan;24(1):25-30. Epub 2013 Oct 1 doi: 10.1016/j.nmd.2013.09.013. PMID: 24239058
Thymectomy in juvenile myasthenia gravis.
Seybold ME, Howard FM Jr, Duane DD, Payne WS, Harrison EG Jr
Arch Neurol 1971 Nov;25(5):385-92. doi: 10.1001/archneur.1971.00490050019001. PMID: 5110884

Clinical prediction guides

Treatment of juvenile myasthenia gravis with tacrolimus: A cohort study.
Wang G, Kessi M, Huang X, Zhang W, Zhang C, He F, Peng J, Yin F, Yang L
Eur J Neurol 2024 Dec;31(12):e16466. Epub 2024 Sep 4 doi: 10.1111/ene.16466. PMID: 39230556Free PMC Article
Juvenile Myasthenia Gravis in North Texas: Clinical Features, Treatment Response, and Outcomes.
Lu M, Reisch J, Iannaccone S, Batley K
Pediatr Neurol 2024 Jul;156:10-14. Epub 2024 Mar 24 doi: 10.1016/j.pediatrneurol.2024.03.019. PMID: 38688232
Genetic, serological and clinical evaluation of childhood myasthenia syndromes- single center subgroup analysis experience in Turkey.
Özsoy Ö, Cinleti T, Günay Ç, Sarıkaya Uzan G, Giray Bozkaya Ö, Çağlayan AO, Hız Kurul S, Yiş U
Acta Neurol Belg 2023 Dec;123(6):2325-2335. Epub 2023 Sep 1 doi: 10.1007/s13760-023-02370-3. PMID: 37656362
Rituximab Therapy in the Treatment of Juvenile Myasthenia Gravis: The French Experience.
Molimard A, Gitiaux C, Barnerias C, Audic F, Isapof A, Walther-Louvier U, Cances C, Espil-Taris C, Davion JB, Quijano-Roy S, Grisel C, Chabrol B, Desguerre I
Neurology 2022 Jun 7;98(23):e2368-e2376. Epub 2022 Mar 21 doi: 10.1212/WNL.0000000000200288. PMID: 35314497
Outcome of children with acetylcholine receptor (AChR) antibody positive juvenile myasthenia gravis following thymectomy.
Heng HS, Lim M, Absoud M, Austin C, Clarke D, Wraige E, Reid C, Robb SA, Jungbluth H
Neuromuscul Disord 2014 Jan;24(1):25-30. Epub 2013 Oct 1 doi: 10.1016/j.nmd.2013.09.013. PMID: 24239058

Recent systematic reviews

The role of thymectomy in the treatment of juvenile myasthenia gravis: a systematic review.
Madenci AL, Li GZ, Weil BR, Zurakowski D, Kang PB, Weldon CB
Pediatr Surg Int 2017 Jun;33(6):683-694. Epub 2017 Apr 11 doi: 10.1007/s00383-017-4086-3. PMID: 28401300

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