Sporadic late onset nemaline myopathy responsive to IVIg and immunotherapy

Margherita Milone, Amiram Katz, Anthony A. Amato, Carl A. Soderland, Miruna Segarceanu, Nathan P. Young, H. Royden Jones

Research output: Contribution to journalArticle

26 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)272-276
Number of pages5
JournalMuscle and Nerve
Issue number2
StatePublished - Feb 1 2010


  • IVIg
  • Monoclonal gammopathy
  • Nemaline myopathy

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)

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    Milone, M., Katz, A., Amato, A. A., Soderland, C. A., Segarceanu, M., Young, N. P., & Jones, H. R. (2010). Sporadic late onset nemaline myopathy responsive to IVIg and immunotherapy. Muscle and Nerve, 41(2), 272-276. https://doi.org/10.1002/mus.21504