Sporadic late onset nemaline myopathy with monoclonal gammopathy of undetermined significance: two cases with long term stability

  • André Truffert | andre.truffert@unige.ch Neuroimmunology and Neuromuscular unit, Department of Clinical Neurosciences, Geneva University Hospital, Geneva, Switzerland.
  • Ruxandra Iancu Ferfoglia Neuroimmunology and Neuromuscular unit, Department of Clinical Neurosciences, Geneva University Hospital, Geneva, Switzerland.
  • Johannes Alexander Lobrinus Department of Pathology, Geneva University Hospital, Geneva, Switzerland.
  • Kaveh Samii Division of Hematology, Geneva University Hospital, Geneva, Switzerland.
  • André Kohler Neuroimmunology and Neuromuscular unit, Department of Clinical Neurosciences, Geneva University Hospital, Geneva, Switzerland.

Abstract

Monoclonal gammopathy of undetermined significance (MGUS) associated to sporadic late onset nemaline myopathy (SLONM) is a rare and severely disabling condition of quickly progressive limb girdle acquired myopathy. It is believed by some authors to be due to myotoxicity of light chain deposits. Two female patients were diagnosed with MGUS associated SLONM. In the first case, diagnosis was delayed by 6 years thus giving time for a severe generalized myopathy and cardiomyopathy to develop. A single anti-myeloma chemotherapy with lenalidomide markedly improved and stabilized the patient’s condition despite respiratory and cardiac insufficiency. In our second patient the condition was identified one year after onset of the first symptom and markedly improved after autologous bone marrow transplantation and lenalidomide. Clinicians should be aware of monoclonal gammopathy associated sporadic late onset nemaline myopathy as this acquired muscle disorder, although extremely rare, may be reversed by adequate management.

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Published
2020-09-16
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Original Articles
Keywords:
sporadic late-onset nemaline myopathy, monoclonal gammopathy of undetermined significance, stem cell transplantation, lenalidomide
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How to Cite
Truffert, A., Iancu Ferfoglia, R., Lobrinus, J. A., Samii, K., & Kohler, A. (2020). Sporadic late onset nemaline myopathy with monoclonal gammopathy of undetermined significance: two cases with long term stability. European Journal of Translational Myology, 30(3). https://doi.org/10.4081/ejtm.2020.9225