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MuSK (Muscle Specific Kinase) Positive Myasthenia: Grave Prognosis or Undue Prejudice?
Annals of Indian Academy of Neurology ( IF 1.7 ) Pub Date : 2020-02-15 , DOI: 10.4103/aian.aian_302_19
Priyanka Samal 1 , Vinay Goyal 1 , Mamta B Singh 1 , M V Padma Srivastava 1
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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.

中文翻译:

麝香(肌肉特异性激酶)阳性肌无力:严重的预后还是过度的偏见?

目的 肌肉特异性激酶 (MusK) 阳性肌无力患者通常被认为预后严重。我们介绍了我们对具有不同抗体状态的肌无力患者的经验。随后是对先前研究的简短讨论以及对 MuSK 阳性肌无力的当前观点,重点关注相关的偏见。材料和方法 本研究在三级中心比较了 23 例 MuSK 阳性肌无力患者、55 例乙酰胆碱受体阳性肌无力患者和 9 例双血清阴性肌无力患者。结果 我们在临床特征、对免疫抑制剂的治疗反应、长期预后和生活质量方面没有发现任何显着差异。结论抗体的血清阳性不应单独用于指导治疗或预测预后。过度的负面预测可能会影响患者的士气。在计划治疗之前,除了抗体状态外,还必须考虑临床特征和对治疗的反应。
更新日期:2020-02-05
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