Skip to main content
Top
Published in: Journal of Neurology 10/2009

01-10-2009 | Letter to the editors

Juvenile seropositive myasthenia gravis with anti-MuSK antibody after thymectomy

Authors: Anna Kostera-Pruszczyk, Hubert Kwiecinski

Published in: Journal of Neurology | Issue 10/2009

Login to get access

Abstract

We report the case of a 17.5-year old girl with generalized myasthenia gravis (MG). When she was 13, she started to complain of episodic diplopia, ptosis and mild fatigability of upper and lower extremity muscles. She was diagnosed with MG 3 years later, after exacerbation of her limb muscle weakness. Acetylcholine receptor antibody was positive (2.56 nmol/l, n < 0.4 nmol/l), anti-MuSK antibodies were not tested at that time. She was treated with a thymectomy. After the operation a gradual worsening of her MG was seen; she reported pirydostygmine intolerance. On retesting, acetylcholine receptor antibodies were negative and she was positive for anti-MuSK antibodies. She received immunosuppressive treatment with prednisone and azathioprine with clinical improvement. She is the third reported patient diagnosed with acetylcholine receptor antibody-positive MG, who became MuSK-positive after thymectomy. Recognition of such clinical patterns may be important for decision making in myasthenia gravis patients.
Literature
1.
go back to reference Anlar B, Senbil N, Kose G, Degerliyurt A (2005) Serological follow-up in juvenile myasthenia: clinical and acetylcholine receptor antibody status of patients followed for at least 2 years. Neuromuscul Disord 15:355–357PubMedCrossRef Anlar B, Senbil N, Kose G, Degerliyurt A (2005) Serological follow-up in juvenile myasthenia: clinical and acetylcholine receptor antibody status of patients followed for at least 2 years. Neuromuscul Disord 15:355–357PubMedCrossRef
2.
go back to reference Evoli A, Tonali PA, Padua L, Monaco ML, Scuderi F, Batocchi AP, Marino M, Bartoccioni E (2003) Clinical correlates with anti-MuSK antibodies in generalized seronegative myasthenia gravis. Brain 126:2304–2311PubMedCrossRef Evoli A, Tonali PA, Padua L, Monaco ML, Scuderi F, Batocchi AP, Marino M, Bartoccioni E (2003) Clinical correlates with anti-MuSK antibodies in generalized seronegative myasthenia gravis. Brain 126:2304–2311PubMedCrossRef
3.
go back to reference Hoch W, McConville J, Helms S, Newsom-Davis J, Melms A, Vincent A (2001) Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies. Nat Med 7:365–368PubMedCrossRef Hoch W, McConville J, Helms S, Newsom-Davis J, Melms A, Vincent A (2001) Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies. Nat Med 7:365–368PubMedCrossRef
4.
go back to reference Kostera-Pruszczyk A, Kaminska A, Dutkiewicz M, Emeryk-Szajewska B, Strugalska-Cynowska MH, Vincent A, Kwiecinski H (2008) MuSK-positive myasthenia gravis is rare in the Polish population. Eur J Neurol 15:720–724PubMedCrossRef Kostera-Pruszczyk A, Kaminska A, Dutkiewicz M, Emeryk-Szajewska B, Strugalska-Cynowska MH, Vincent A, Kwiecinski H (2008) MuSK-positive myasthenia gravis is rare in the Polish population. Eur J Neurol 15:720–724PubMedCrossRef
5.
go back to reference Limburg PC, The TH, Hummel-Tappel E, Oosterhuis HJ (1983) Anti-acetylcholine receptor antibodies in myasthenia gravis. Part 1. Relation to clinical parameters in 250 patients. J Neurol Sci 58:357–370PubMedCrossRef Limburg PC, The TH, Hummel-Tappel E, Oosterhuis HJ (1983) Anti-acetylcholine receptor antibodies in myasthenia gravis. Part 1. Relation to clinical parameters in 250 patients. J Neurol Sci 58:357–370PubMedCrossRef
6.
go back to reference Murai H, Noda T, Himeno E, Kawano Y, Ohyagi Y, Shiraishi H, Motomura M, Kira J (2006) Infantile onset myasthenia gravis with MuSK antibodies. Neurology 67:174PubMedCrossRef Murai H, Noda T, Himeno E, Kawano Y, Ohyagi Y, Shiraishi H, Motomura M, Kira J (2006) Infantile onset myasthenia gravis with MuSK antibodies. Neurology 67:174PubMedCrossRef
7.
go back to reference Sanders DB, El Salem K, Massey JM, McConville J, Vincent A (2003) Clinical aspects of MuSK antibody positive seronegative MG. Neurology 60:1978–1980PubMed Sanders DB, El Salem K, Massey JM, McConville J, Vincent A (2003) Clinical aspects of MuSK antibody positive seronegative MG. Neurology 60:1978–1980PubMed
8.
go back to reference Sanders DB, Juel VC (2008) MuSK-antibody positive myasthenia gravis: questions from the clinic. J Neuroimmunol 201–202:85–89PubMedCrossRef Sanders DB, Juel VC (2008) MuSK-antibody positive myasthenia gravis: questions from the clinic. J Neuroimmunol 201–202:85–89PubMedCrossRef
9.
go back to reference Saulat B, Maertens P, Hamilton WJ, Bassam BA (2007) Anti-musk antibody after thymectomy in a previously seropositive myasthenic child. Neurology 69:803–804PubMedCrossRef Saulat B, Maertens P, Hamilton WJ, Bassam BA (2007) Anti-musk antibody after thymectomy in a previously seropositive myasthenic child. Neurology 69:803–804PubMedCrossRef
10.
go back to reference Strobel P, Moritz R, Leite MI, Willcox N, Chuang WY, Gold R, Nix W, Schalke B, Kiefer R, Muller-Hermelink HK, Jaretzki IA, Newsom-Davis J, Marx A (2008) The ageing and myasthenic thymus: a morphometric study validating a standard procedure in the histological workup of thymic specimens. J Neuroimmunol 201–202:64–73PubMedCrossRef Strobel P, Moritz R, Leite MI, Willcox N, Chuang WY, Gold R, Nix W, Schalke B, Kiefer R, Muller-Hermelink HK, Jaretzki IA, Newsom-Davis J, Marx A (2008) The ageing and myasthenic thymus: a morphometric study validating a standard procedure in the histological workup of thymic specimens. J Neuroimmunol 201–202:64–73PubMedCrossRef
11.
go back to reference Vincent A, Leite MI, Farrugia ME, Jacob S, Viegas S, Shiraishi H, Benveniste O, Morgan BP, Hilton-Jones D, Newsom-Davis J, Beeson D, Willcox N (2008) Myasthenia gravis seronegative for acetylcholine receptor antibodies. Ann NY Acad Sci 1132:84–92PubMedCrossRef Vincent A, Leite MI, Farrugia ME, Jacob S, Viegas S, Shiraishi H, Benveniste O, Morgan BP, Hilton-Jones D, Newsom-Davis J, Beeson D, Willcox N (2008) Myasthenia gravis seronegative for acetylcholine receptor antibodies. Ann NY Acad Sci 1132:84–92PubMedCrossRef
Metadata
Title
Juvenile seropositive myasthenia gravis with anti-MuSK antibody after thymectomy
Authors
Anna Kostera-Pruszczyk
Hubert Kwiecinski
Publication date
01-10-2009
Publisher
D. Steinkopff-Verlag
Published in
Journal of Neurology / Issue 10/2009
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-009-5215-2

Other articles of this Issue 10/2009

Journal of Neurology 10/2009 Go to the issue