Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Muscle Nerve. 2010 Feb;41(2):272-6. doi: 10.1002/mus.21504.
Sporadic late onset nemaline myopathy (SLONM) is a progressive myopathy of indeterminate etiology and poor outcome. If associated with a monoclonal gammopathy, SLONM carries a more unfavorable prognosis. Immunotherapy was unsuccessful. We report two HIV-negative SLONM/monoclonal gammopathy patients who improved following intravenous immunoglobulin (IVIg) treatment alone or in combination with immunosuppressant agents. This favorable response to treatment suggests that a dysimmune mechanism is operative in some SLONM individuals. We suggest that IVIg deserves initial consideration for SLONM therapy.
散发性迟发性先天性肌营养不良症（SLONM）是一种病因不明、预后不良的进行性肌病。如果与单克隆丙种球蛋白血症相关，SLONM 的预后更差。免疫疗法无效。我们报告了两名 HIV 阴性的 SLONM/单克隆丙种球蛋白血症患者，他们单独或联合免疫抑制剂治疗后病情有所改善。这种对治疗的良好反应表明，在某些 SLONM 患者中存在免疫失调机制。我们建议 IVIg 应作为 SLONM 治疗的首选。
