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. 2020 Jan-Feb;23(1):32-37.
doi: 10.4103/aian.AIAN_302_19.

MuSK (Muscle Specific Kinase) Positive Myasthenia: Grave Prognosis or Undue Prejudice?

Affiliations

MuSK (Muscle Specific Kinase) Positive Myasthenia: Grave Prognosis or Undue Prejudice?

Priyanka Samal et al. Ann Indian Acad Neurol. 2020 Jan-Feb.

Abstract

Objectives: Patients with muscle-specific kinase (MuSK)-positive myasthenia are generally considered to have a grave prognosis. We present our experience of patients with myasthenia with different antibody status. This is followed by a short discourse on previous studies and the current view on MuSK-positive myasthenia, focusing on the associated prejudice.

Materials and methods: This study compares 23 patients with MuSK-positive myasthenia with 55 patients with acetylcholine receptor-positive myasthenia and 9 patients with double-seronegative myasthenia at a tertiary level center.

Results: We did not find any significant difference in terms of clinical characteristics, treatment response to immunosuppressants, long-term prognosis, and quality of life.

Conclusion: Seropositivity for antibodies should not be used in isolation to guide the management or predict the prognosis. Undue negative prognostication may affect the morale of patient. Clinical features and response to therapy in addition to antibody status must be considered before planning therapy.

Keywords: MuSK; myasthenia; prognosis; quality of life; rituximab.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Comparison of MGFA class at onset, maximum severity, and at last follow-up. Onset MGFA P = 0.59, max MGFA P = 0.63, last follow-up MGFA P = 0.91 AChR-MG: acetylcholine receptor–positive antibody myasthenia gravis; DN-MG: double-seronegative myasthenia gravis; MGFA: Myasthenia Gravis Foundation of America; MuSK-MG: muscle-specific kinase myasthenia gravis

References

    1. Hoch W, McConville J, Helms S, Newsom-Davis J, Melms A, Vincent A. Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies. Nat Med. 2001;7:365–8. - PubMed
    1. Guptill JT, Sanders DB. Update on muscle-specific tyrosine kinase antibody positive myasthenia gravis. Curr Opin Neurol. 2010;23:530–5. - PubMed
    1. Díaz-Manera J, Martínez-Hernández E, Querol L, Klooster R, Rojas-García R, Suárez-Calvet X, et al. Long-lasting treatment effect of rituximab in MuSK myasthenia. Neurology. 2012;78:189–93. - PubMed
    1. Burns TM, Sadjadi R, Utsugisawa K, Gwathmey KG, Joshi A, Jones S, et al. International clinimetric evaluation of the MG-QOL15, resulting in slight revision and subsequent validation of the MG-QOL15r. Muscle Nerve. 2016;54:1015–22. - PubMed
    1. Lee JY, Sung JJ, Cho JY, Oh DH, Kim HJ, Park JH, et al. MuSK antibody-positive, seronegative myasthenia gravis in Korea. J Clin Neurosci Off J Neurosurg Soc Australas. 2006;13:353–5. - PubMed