Journal of Shandong University (Health Sciences) ›› 2022, Vol. 60 ›› Issue (10): 74-81.doi: 10.6040/j.issn.1671-7554.0.2022.0084

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Three cases of sporadic late-onset nemaline myopathy with monoclonal gammopathy and literature review

DONG Wenhao1, ZHAO Bing2, LI Zhao1, ZHANG Chen1, YUAN Chenglu1   

  1. 1. Department of Hematology;
    2. Department of Neurology, Qilu Hospital(Qingdao), Cheeloo College of Medicine, Shandong University, Qingdao 266035, Shandong, China
  • Published:2022-09-30

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Abstract: Objective Three patients suspected of sporadic late-onset nemaline myopathy with monoclonal gammopathy were diagnosed by muscle pathological examination and monoclonal protein screening, so as to improve the understanding of this rare disease and explore its diagnosis and treatment. Methods The characteristics of this disease were summarized and relevant literatures were reviewed. Results All of the 3 patients presented with progressively aggravated muscle weakness, which was diagnosed by muscle biopsy as sporadic late-onset nemaline myopathy with monoclonal gammopathy. Autologous hematopoietic stem cell transplantation was effective. Conclusion Sporadic late-onset nemaline myopathy is a rare, subacute progressive myopathy characterized by proximal muscle weakness and atrophy, often complicated with monoclonal gammopathy. Therapies directed at eradication of M protein are effective.

Key words: Monoclonal gammopathy, Sporadic late-onset nemaline myopathy, Autologous hematopoietic stem cell transplantation

CLC Number: 

  • R746.9
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