Unusual presentation double seronegative Myasthenia gravis
Abstract
ABSTRACT
We reported a case of 22-year-old male patient with swallowing difficultes and double vision. Diagnosed on the basis of positive pharmacological tests , damage to the postsynaptic neuromuscular junction and computed tomography of the mediastinum. Antibodies to acetylcholine receptors and antibodies to muscle specific tyrosine kinase were negative . Partial improvement of neurological findings on therapy was administrated pyridostigmine bromide, cyclosporine, methylprednisolone. The patient has responded favorably to plasma exchange with the withdrawal of all clinical symptoms and that confirmation the hypothesis of the humoral factors in the pathogenesis of double-negative of MG.
Key words: double seronegative Myasthenia gravis, autoantibodies
References
F Romi, J.A.Aarli and N.E. Gilhus. Seronegative myasthenia gravis: disease severity and prognosis. European Journal of Neurology 2005; 12: 413-418.
A.Vincent, J.Bowen, J.Newsom-Davis and J.McConvillie. Seonegative generalized mysthenia gravis: clinical features, antibodies and their target. Lancet Neurology 2003; 2: 99-106.
Pevzner A, Schoser B, Peteres K, Cosma NC, Karakatsani A, Shalke B, Melms A, Kroger S. Anti-LRP4 autoantibodies in AchR- and MuSK antibody negative myasthenia gravis. J Neurol 2012; 259(3): 427-35.
Liyanage Y, Hoch W, Beeson D, Vincet A. The agrin/muscle specific kinase pathway. New target for autoimmune and genetic disorders at the neuromuscular junction. Muscle Nerve 2002; 25: 4-16.
Farrugia ME, Vincent A. Autoimmune mediated neuromuscular junction defects. Curr Opin Neurol 2010; 23: 489-495.
Evoli A, Tonali P, Padua L, Monaco M, Scuderi F, Batocchi A, Marino M, Bartoccioni E. Clinical correlates with anti MuSK antibodies in generalized seronegative myasthenia gravis. Brain 2003; 126: 2304-2311.
Kim N, Stiegler AL, Cameron TO, Hallock PT, Gomez AM, Huang JH, Hubbard SR, Dustin ML, Burden SJ. LRP 4 is a receptor for agrin and forms a complex with MuSK. Cell 2008; 135:334-342.
Higuchi O, Hamuro J, Motomura M, Yamanashi Y. Autoantibodies to low-denssity lipoprotein receptor-related protein 4 in myasthenia gravis. Ann Neurol 2011; 69: 418-422.
Simon-Chazottes D, Tutois S, Kuehn M, Evans M, Bourgade F, Cook S, Davisson MT, Guénet JL.. Mutations in the gene encoding the low-density lipoprotein LRP4 cause abnormal limb development in the mouse. Genomics 2006; 87: 673-677.
Courtney M. Karner, Martin F. Dietrich, Eric B. Johnson, Natalie Kappesser, Christian Tennert, Ferda Percin, Bernd Wollnik, Thomas J. Carrill and Joachim Herz. LRP4 regulates initiation of ureteric budding and is crucial for kidney formation of the neuromuscular junction. Development 2010; 133: 4993-5000.