Clinical features of myasthenia related with autoantibodies to muscle specific tyrosine kinase (MuSK)

Sanadze A.G.; Sidnev D.V.; Karganov M.Iu.

doi:https://doi.org/

Sanadze A.G.

GKB #51, Moskovskiĭ miastenicheskiĭ tsentr

Sidnev D.V.

GKB #51, Moskovskiĭ miastenicheskiĭ tsentr

Karganov M.Iu.

NII obshcheĭ patologii i patofiziologii RAMN, Moskva

Clinical features of myasthenia related with autoantibodies to muscle specific tyrosine kinase (MuSK)

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Sanadze A.G., Sidnev D.V., Karganov M.Iu.

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Journal: S.S. Korsakov Journal of Neurology and Psychiatry. 2010;110(12): 72‑76

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Sanadze AG, Sidnev DV, Karganov MIu. Clinical features of myasthenia related with autoantibodies to muscle specific tyrosine kinase (MuSK). S.S. Korsakov Journal of Neurology and Psychiatry. 2010;110(12):72‑76. (In Russ.)

Подписка 2026 Журнал неврологии и психиатрии им. С.С. Корсакова (S.S. Korsakov Journal of Neurology and Psychiatry)

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Abstract:

Anti-acetylcholine receptor antibodies are detected in 80 to 90% of patients with generalized myasthenia gravis - seropositive myasthenia gravis (SPMG). Approximately 10% to 20% of patients with autoimmune MG do not have antibodies to acetylcholine receptor (AChR) - seronegative MG (SNMG). An immunological study of the blood serum of patients with SNMG revealed patients with or without antibodies to muscle specific tyrosine kinase (MuSK+ and MuSK–, respectively). A clinical study of 13 MuSK+ patients with myasthenia showed that this group was characterized by predominant affect of mimic and bulbar muscles with the rare involvement of eye movement and body muscles. In Musk+ patients with myasthenia, the clinical efficacy of antitoxin esterase drugs was minimal or absent. No unequivocal conclusion on the clinical pattern of this form of myasthenia has been made.

Keywords:

seronegative myasthenia

acetylcholine receptors

muscle specific tyrosine kinase

Authors:

Sanadze A.G.

GKB #51, Moskovskiĭ miastenicheskiĭ tsentr

Sidnev D.V.

GKB #51, Moskovskiĭ miastenicheskiĭ tsentr

Karganov M.Iu.

NII obshcheĭ patologii i patofiziologii RAMN, Moskva

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References:

Sidnev D.V., Karganov M.Yu., Shcherbakova N.I. i dr.Antitela k atsetilkholinovomu retseptoru u bol'nykh s razlichnymi klinicheskimi formami miastenii i miastenicheskim sindromom Lamberta-Itona. Zhurn nevrol i psikhiat 2006; 106: 1: 53-55.
Shcherbakova N.I., Sanadze A.G., Sidnev D.V., Rudnichenko V.A.Klinicheskie i elektrofiziologicheskie osobennosti seronegativnoi miastenii. Zhurn nevrol i psikhiat 2008; 108: 3: 4-10.
Bartoccione E., Scuderi F., Minicuci G.M. et al.Anti MuSK antibodies: correlation with myasthenia gravis severity. Neurology 2006; 67: 505-507.
Benveniste O., Jacobson L., Farrugia M.E. et al.MuSK antibody-positive myasthenia gravis plasma modifies MURF-1 expression in C2C12 cultures and mouse muscle in vivo. J Neuroimmunol 2005; 170: 41-48.
Boneva N., Frenkian-Cuvelier M., Bidault J. et al.Major pathogenic effects of anti MuSK antibodies in myasthenia gravis. J Neuroimmunol 2006; 177: 119-131.
Caress J.B., Hunt C.H., Batish S.D.Anti- MuSK myasthenia gravis presenting with purely ocular findings. Arch Neurol 2005; 62: 1002-1003.
Deymeer F., Gungor-Tuncer O., Yilmaz V. et al.Clinical comparison of anti-MuSK and anti-AChR - positive and seronegative myasthenia gravis. Neurology 2007; 68: 609-611.
Evoli A., Bianchi M.R., Riso R. et al.Response to therapy in myasthenia gravis with anti-MuSK antibodies. Ann NY Acad Sci 2008; 1132: 76-83.
Evoli A., Tonali P.A., Padua L. et al.Clinical correlates with anti MuSK antibodies in generalized seronegative myasthenia gravis. Brain 2003; 126: 2304-2311.
Farrugia M.E., Kennett R.P., Newsom-Davis J. et al.Single-fiber electromyography in limb and facial muscles in muscle-specific kinase antibody and acetylcholine receptor antibody myasthenia gravis. Muscle & Nerve 2006; 33: 568-570.
Farrugia M.E., Kennett R.P., Hilton-Jones D. et al.Quantitative EMG of facial muscles in myasthenia patients with MuSK antibodies. Clin Neurophysiol 2007; 118: 269-277.
Farrugia M.E., Robson M.D., Clover L. et al.MIR and clinical studies of facial and bulbar involvement in MuSK antibody-associated myasthenia gravis. Brain 2006; 129: 1481-1492.
Hanisch F., Eger K., Zierz S.MuSK antibody-positive pure ocular myasthenia gravis. J Neurol 2006; 253: 659-660.
Hatanaka Y., Hemmi S., Morgan M.B. et al.Nonresponsiveness to anticholinesterase agents in patients with MuSK antibody-positive MG. Neurology 2005; 65: 1508-1509.
Hoch W., McConville J., Helms S. et al.Autoantibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies. Nat Med 2001; 7: 365-368.
Jha S., Xu K., Maruta T. et al.Myasthenia gravis induced in mice by immunization with the recombinant extracellular domain of rat muscle-specific kinase (MuSK). J Neuroimmunol 2006; 175: 107-117.
Kuwabara S., Nemoto Y., Misawa S. et al.Anti-MuSK positive myasthenia gravis: neuromuscular transmission failure in facial and limb muscles. Acta Neurol Scand 2007; 115: 126-128.
Lauriola L., Ranelletti F., Maggiano N. et al.Thymus changes in anti- MuSK - positive and negative myasthenia gravis. Neurology 2005; 64: 536-538.
Lavrnic D., Losen M., Vujic A. et al.The features of myasthenia gravis with auto antibodies to MuSK. J Neurol Neurosurg Psychiat 2005; 76: 1099-1102.
Lee J.Y., Sung J.J., Cho J.Y. et al.MuSK antibody-positive seronegative myasthenia gravis in Korea. J Clin Neurosci 2006; 13: 353-355.
Letie M.I., Strobel P., Jones M. et al.Fever thymic changes in MuSK antibody-positive than in in MuSK antibody-negative MG. Ann Neurol 2005; 57: 444-448.
McConville J., Farrugia M.E., Beeson D. et al.Detection and characterization of MuSK antibodies in seronegative myasthenia gravis. Ann Neurol 2004; 55: 580-584.
NemotoY., Kuwabara S., Misawa S. et al.Patterns and severity of neuromuscular transmission failure in seronegative myasthenia gravis. J Neurol Neurosurg Psychiat 2005; 76: 714-718.
Niks E.H., Kuks J.B.M., Verschuuren J.J.G.M.Epidemiology of myasthenia gravis with anti muscle specific kinase antibodies in the Netherlands. J Neurol Neurosurg Psychiat 2007; 78: 417-418.
Oh S.J., Hatanaka Y., Hemmi S. et al.Repetitive nerve stimulation of facial muscles in MuSK antibody-positive myasthenia gravis. Muscle & Nerve 2006; 33: 500-504.
Padua L., Tonali P.A., Aprile I. et al.Seronegative myasthenia gravis: comparison of neurophysiological picture in MuSK+ and MuSK– patients. Eur J Neurol 2006; 13: 273-276.
Pasnoor M.E., Wolfe G., Nations S. et al.Clinical findings in MuSK antibody-positive myasthenia gravis. Muscle & Nerve 2010; 41: 370-374.
Punga A.R., Flink R., Askmark H. et al.Cholinergic neuromuscular hyperactivity in patients with myasthenia gravis seropositive for MuSK antibody. Muscle & Nerve 2006; 34: 111-115.
Romi F., Aarli J.A., Gilhus N.E.Seronegative myasthenia gravis: disease severity and prognosis. Eur J Neurol 2005; 12: 413-418.
Sanders D.B., El-Salem K., Massey J.M. et al.Clinical aspects of MuSK antibody-positive seronegative Mg. Neurology 2003; 60: 1978-1980.
Selcen D., Fukuda T., Shen X.-M. et al.Are MuSK antibodies the primary cause of myasthenic symptoms? Neurology 2004; 62: 1945-1950.
Shigemoto K., Kubo S., Maruyma N. et al.Induction of myasthenia by immunization against muscle-specific kinase (MuSK). J Neuroimmunol 2006; 116: 1016-1024.
Shiraishi H., Motomura M., Yoshimura T. et al.Acetylcholine receptor loss and postsynaptic damage in MuSK antibody-positive myasthenia gravis. Ann Neurol 2005; 57: 289-293.
Stickler D.E., Massey J.M., Sanders D.B.MuSK antibody-positive myasthenia gravis: clinical and electrodiagnostic patterns. Clin Neurophisiol 2005; 116: 2065-2068.
Vincent A., Letie M.I.Neuromuscular junction autoimmune disease: muscle-specific tyrosine kinase antibodies and treatment myasthenia gravis. Curr Opin Neurol 2005; 18: 519-525.
Vincent A., McConville J., Farrugia M.E., Newsom-Davis J.Seronegative myasthenia gravis. Semin Neurol 2004; 24: 125-133.
Wolfe G.I., Oh S.J.Clinical phenotype of muscle-specific tyrosine kinase antibody-positive myasthenia gravis. Ann NY Acad Sci 2008; 1132: 71-75.
Yeh J.-H., Chen W.-H., Chiu H.-C. et al.Low frequency of MuSK antibody in generalized seronegative myasthenia gravis among Chinese. Neurology 2004; 62: 2131-2132.
Zhou I., McConville J., Chaudhry V.Clinical comparison of muscle-specific tyrosine kinase (MuSK) antibody-positive and -negative myasthenic patients. Muscle & Nerve 2004; 30: 55-60.